elitoki
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Hi, I need some advice how can I better my scores in endo and rheumatology. Should I have to try other sources of books for the prep? I read few times K-note and FA, but I'm still getting wrong some complicated(?) Q.
What should I have to do?
Thanks~
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| neuroblastoma
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learn from these wrong complicated questions... no matter wht
read UW explnations carefully+ re-check things from kap notes if forgetting
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yahoo mesenger and skype ID asdvsd2003
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| drtanvir
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REHUMATOID ARTHERITIS: Affects 0.03%-1.5% of population. Most of white patients with RA have MHC class II type HLA-DR4 or DR1 or both. The concardence of RF +ive RA is increased 6times among dizygotic twins. The risk of RA in MZ twins is increased 30times when sibling has the disease.
The joints most commonly involved in RA are the MCP, PIP, wrist and MTP joints. The distribution of involvement is symmetrical and polyarticular, predominantly small joints are involved.
Hallmark of joint inflammation: stiffness, heat, redness, soft tissue swelling, pain and dysfunction.
Musculoskeletal complications:
A. Cervical supine: Half of all patients with chronic RA have radiographic involvement of the atlantoaxial joint. Patients with cervical spine involvement may present with occipital pain, sgns of myleopathy, weakness and paresthesias of the hands and drop attacks.
Indications for surgical treatment: neurologic or vascular compromise or intractable pain. The probabililty of cervical involvement is predicted by the severity of peripheral arthritis.
B. Popletial cyst: Also called Bakers cyst. Rupture of popletical cyst may resemble acute trhombophelbitis(pseudophelibits). Fever, leukocytosis and ecchymosis around the ankle(cressent sign) can occur with rupture.
Ultrasonography can distinguish a cyst from popletial artery aneurysm, lymphoadenopathy, phlebitis and tumor.
Treatment of popletial cyst requires improvement in knee arthritis.
C. tenocynovitis: of the finger flexor and extensor tendon sheath is common and lead to tendon rupture. Other tenosynovitis syndrome in RA include de Quervian and wrist tenosynovits. Treatment of acute tenosynovitis include immobilization, warm soaks, NSAIDs and local corticosteroids injections in the sheath.
D. Carpel tunnel syndrome: RA is common cause of carpel tunnel syndrome- parestheisas of the hand in a typical median nerve distribution.
Treatment: resting splints, control of inflammation and local corticosteroids injections. Surgical release for persistent symptoms.
Extraarticular complications: occurs exclusively in seropositive RA.
Rehumatoid nodules: MC extraarticular complication. Occurs over extensor surfaces and at pressure points and are prone to ulceration and infection.
Rehumatoid vasculitis: occurs in the setting of severe, deforming arthritis and a high titre of RF. Rheumatoid vasculitis is mediated by the deposition of circulating immune complexes in the blood vessel walls.rehumatoid vasculitis comprises a spectrum of vascular disease including rheumatoid nodules and oblitirative endarteriopathy, leukocytoclastic small vessel vascultis(produces palpable purpura or cutenous ulceration, particularly over the malleoli of lower extremities). And secondary polyarteritis(clinically and histopathologicaly identical to PN, results in mononeuritis multiplex).
Neurological manifestations: mild peripheral sensory neuropathy, painful sensory-motor neuropathy suggests vasulitis or nerve entrappement. Cervical vertebral sublaxation can cause mylopathy. Erosive changes may promote the bassilr inviaginatin of the odontoid process of C2 into underside of the brain→spinal cord compression which may lead to death.
Pulmonary manifestations: Rehumatoid pulmonary disease is common but asymptomatic until pleural effusion interfere with respiration.
The exudative pleural fluid is remarkable for low level of glucose. High resolution CT helps to distinguish among rheumatoid associated interstitial lung disease, including interstitial pneumonitis, interstitial fibrosis and bronchiolitis oblitirans with or without organizing pneumonia.
MTX hypersensitivity pneumonitis may be life threatening complication of therapy.
Cardiac abnormalities: Patients rarely present with acute pericardial symptoms despite frequent serous pericarditis.
Rheumatoid pericardial disease frequently presents with edema or ascities due to occult constrictive disease. Chronic constrictive disease necessitates surgical treatment.
Liver abnormalities: increased level of liver enzymes, particularly alkaline phosphatase may occur in RA. Nodular hyperplasia of the liver can complicate RA and leads portal hypertension and hypersplenism.
Ophthalmic abnormalities: keratoconjuctivitis siccia or secondary Sjogran syndrome is the most common ophthalmic complication in RA. Sever scleritis progressive to scleromalacia perforans causes blindness.
Labs: Normocytic anemia, leukocytois, thrombocytosis and hypoalbuminemia.
RF not specific for RA. Anti CCP(citulinated protein) antibodies may present when RF is absent, are equal sensitive and more specific than RF.
Synovial fluid- cloudy, light yellow, poor viscosity and contains 10,000 to 75,000 leukocytes/µl predominantly neutrophils.
The characteristic radiological changes: periarticular osteoporosis, systemic narrowing of joint space and bony erosion of joints of margins. These occur earliest in the hand and MTP joints.
Natural history of RA:
In a third of patient the onset of RA is rapid(days or weeks).
Among the patients with RA 70-90%have persistant chronic progressive arthritis.
The relationship between disease duration and inability to work is nearly linear.
Treatment: Goal of management include relieve inflammation and pain and maintaining function. A disease modifying regimen is started when RA is diagnosed. Low dose prednisone may be necessary to preserve function during initiation of DMARD therapy. With disease modifying agents uninterrupted treatment for 3-6 months is needed to assess efficacy.
Surgery in treatment: orthopedic surgery is most important advance in treatment of medical resistant RA.
Total arthoplasty has a slight poorer long term outcome in RA than OA.
Conditions related to RA: seronegative RA is not associated with extraarticular manifestations.
Seronagative arthritis in elderly: In patients older than 60 years, milder arthritis and polyarticular inflammation which suddenly develops and is controlled best with low dose prednisone. AntiCCP antibodies may be helpful in distinguishing this condition from polymayalgia rehumatica. Some elderly patients with seronegative arthritis present with acute polyartheritis and pitting edema of the hands and feet so called (RS3PE)—Rx with low dose prednisone.
Adult onset Still disease: a patient present with classical triad of fever, rash and arthritis.
RF and ANA are absent in Still disease. There is predilection of wrists, shoulders, hips and knees. Sore throat occurs in 60% of patients .
Rx.. High dose aspirin or indomethacin, corticosteroids and MTX.
Feltys syndrome: occurs in <1% of patients with sero +ive RA (12 yrs and more). Classic triad of RA, leucopenia and splenomegaly is present. High titre of RF is the rule.
Pts with Felty syndrome most often die of infections.
Rx. Corticosteroids, MTX , G-CSF and splenomegaly.
Sjoggren syndrome: classical triad- keratoconjuctivits sicca(with or without lacrimal gland enlargement), xerostomia(with or without salivary gland enlargement) and connective tissue disease (usually RA).
Histologicaly CD4 lymphocyte infiltration and destruction of lacrimal and salivary glands characterize it. Mst of patients have polyclonal hyper gmmaglobulinemia, SSA, and SSB. Patients with Sjoggren syndrome but not with seronagative sicca syndrome are at increased risk for non Hodgkin lymphoma. Lip biopsy is the only specific diagnostic technique. It is a simple procedure with minimal risk involved. The diagnosis is confirmed when the biopsy shows lymphoid foci in accessory salivary glands. Schirmer test measures the quantity of tears secreted. A Whatman filter paper is placed in the lower conjunctival sac and the tear formation is assessed based on the amount of wetness. A positive test presents as less than 5 mm wetness after 5 minutes. However, this is not a specific test.
Rx. Control of inflammation and symptoms of dryness. Pilocorpine- 5mg orally 4times daily. SE. flushing and sweating.
A Sjoggren like syndrome hase been described in HIV infection.
Reiter’s syndrome: consists of urethritis, conjunctivitis, mucocutaneous lesions and arthritis. It usually follows dysenteric infection or sexually-transmitted infection. The mucocutaneous lesions may be in the form of stomatitis, balanitis or keratoderma blennorrhagicum. Typical skin lesion of Reiter’s syndrome is Keratoderma blennorrhagicum found on palms and soles. These lesions present as clear vesicles on red bases and later on develop into macules or papules. They resemble very closely to pustular psoriasis. Other skin lesions of Reiter’s syndrome are circinate balanitis that are painless shallow ulcers present on the glans penis or urethral meatus.
Behçet’s syndrome: is a multi-systemic inflammatory condition characterized by recurrent oral and genital ulcers, skin lesions; seen most commonly in the Turkish, Asian and Middle Eastern population.
The international criteria for diagnosis includes: Recurrent oral ulcers plus two of the following features - recurrent genital ulcers; eye lesions, including anterior uveitis and posterior uveitis; retinal vascularization; skin lesions including erythema nodosum, acneiform nodules and papulopustular lesions; and a positive pathergy test. Some patients have involvement of the gastrointestinal, skeletal and vascular systems. Combined environmental and hereditary factors are assumed to play a role in the pathophysiology of this condition. Corticosteroids offer relief from most of the symptoms but do not protect from progression to dementia or blindness.
Osteoarthritis: is a disorder characterized by progressive deterioration and loss of articular cartilage. It can affect any joint, but larger joints are most affected. With progressive disease, joint motion becomes limited. Osteoarthritis classically involves distal interphalangeal joints.
Acetaminophen is the initial therapy to relieve pain in patients of osteoarthritis. If analgesics, like acetaminophen, fail to control pain NSAIDs, like indomethacin, are indicated. Cyclooxygenase-2 (COX-2) inhibitors, like celecoxib, are indicated for the treatment of osteoarthritis when patient is at risk for upper GI bleed and has failed to respond to analgesics. Weight reduction and exercise program only complement drug treatment. Exercise program is meant to improve functional status of the patient. Weight reduction may improve the symptoms of knee osteoarthritis, but this patient is not obese and his symptoms are not too severe.
Ankylosing spondylitis: typical features include back pain with morning stiffness which improves with exercise, insidious onset, presence of symptoms for more than three months, positive family history, reduced range of forward flexion of the lumber spine on Schober testing and reduced chest expansion. Diagnosis of ankylosing spondylitis can’t be made unless there is evidence of sacroiliitis that is essential for the diagnosis. This is the earliest change to be seen radiographically. Therefore, when a patient has typical features of ankylosing spondylitis plain X-ray of the sacroiliac joint is the next step to establish the diagnosis. Plain X-ray of the spine shows evidence of sacroiliitis in most cases and establishes the diagnosis. Other radiographic abnormalities suggesting the diagnosis of ankylosing spondylitis include erosions of the ischial tuberosity and iliac crest. Early in the course of disease squaring of the vertebral disease on plain X-ray may also suggest the diagnosis.
When ankylosing spondylitis is strongly suspected clinically and radiographic findings of plain X-ray are negative or equivocal, CT scan is indicated to establish the diagnosis. MRI may also be used for this purpose but CT scan is usually recommended in such setting.
ANA and rheumatoid factor are absent in ankylosing spondylitis and other seronegative spondyloarthropathies.
HLA-B27 is frequently (>90% of the patients) present in patients of ankylosing spondylitis and other spondyloarthropathies. Its presence is not specific for the diagnosis of ankylosing spondylitis but if it is found to be negative, then the diagnosis of ankylosing spondylitis is unlikely.
Uveitis is the most frequent extra-articular manifestation of ankylosing spondylitis and it presents with unilateral eye pain, blurred vision and photophobia. Diagnostic finding, which distinguishes anterior uveitis from other causes of red eye, is presence of pus in the anterior chamber on slit lamp eye examination. Another important slit lamp finding is presence of haze in the anterior chamber. Uveitis is usually unilateral and injection is perilimbal.
Regular exercise has been shown to halt the progression of ankylosing spondylitis. The principal goal of management is participation in exercise program that increases mobility, preserves range of motion and reduces the development of severe deformity. Bed rest on the other hand will decrease the functional capacity and will lead to disability and deformities and should be discouraged.
Drug treatment alone has not been shown to retard the progression of disease. NSAIDs are used for pain control that enables patients to maintain their functional activity and to participate in exercise program.
Sulfasalazine is effective for symptom control of peripheral joint involvement in ankylosing spondylitis. Its role in halting the disease progression is not established.
Oral steroids don’t provide symptomatic benefit. Intraarticular injections of steroids provide symptomatic benefit and may be used when disease is refractory to NSAIDs.
Surgery is indicated only when disease is severe and refractory to medical treatment.
Cervical spondylosis: It is estimated that cervical spondylosis affects 10% of people older than 50 years of age. The history of chronic neck pain is typical. Limited neck rotation and lateral bending is due to osteoarthritis and secondary muscle spasm. Sensory deficit is due to osteophyte-induced radiculopathy and isolated sensory abnormalities are associated with good prognosis. Typical radiographic findings include bony spurs and sclerotic facet joints. Interestingly, such ‘osteoarthritic’ changes are common in asymptomatic patients older than 50 years of age; therefore, specificity of these findings are low. Other findings during cervical spondylosis may include narrowing of the disk spaces and hypertrophic vertebral bodies.
Relapsing polychondritis: is an idiopathic disorder characterized by recurrent inflammation of cartilaginous structures and other internal organs. Multiple organs may be involved in this disorder but involvement of ear is the most frequent. Other organs involved include eyes, joints, vascular system, skin and nervous system. Unilateral or bilateral inflammation of auricle occurs with sparing of the non-cartilaginous ear lobules and produces marked pain and tenderness. Skin of the auricle becomes violaceous or erythematous. There may be repeated episodes producing deformity of the auricle. Eye involvement is usually in the form of conjunctivitis, episcleritis or scleritis.
Psoriatic arthritis: About 70% of patients with psoriasis have asymmetric oligoarticular arthritis, affecting two to three joints simultaneously. The proximal joints of the hands and feet are frequently affected. Sausage-shaped digits may be present. The arthritis can be deforming and destructive. Sacroiliitis and spondylitis may also occur. Most patients have an associated patchy skin rash over the elbows, knees or trunk. You do not see nodules in psoriasis.
Recognize the features of psoriatic arthritis. It can present in 5 different forms.
1. Distal interphalangeal (DIP) joint involvement.
2. Asymmetric oligoarthritis.
3. Symmetric polyarthritis, similar to rheumatoid arthritis.
4. Arthritis mutilans, characterized by deforming and destructive arthritis.
5. Spondyloarthropathy, including both sacroiliitis and spondylitis.
These patients often have nail changes (pitting nails and onychodystrophy). Majority patients with psoriatic arthritis typically have skin lesions for years before the joint involvement. However, 15% of patients may develop joint disease prior to skin disease. DIP involvement and the presence of nail changes may be the only clue in these patients. X-ray of the joints shows both combination of erosion and bone growth. Other changes include pencil cup deformity of fingers, fluffy periostitis, and bilateral asymmetrical fusiform soft tissue swelling.
These patients need treatment for the arthritis and the skin lesions, preferably with an agent that treats both and thus ensures compliance of the patient to the treatment regimen (complicated treatment protocols with multiple oral and topical medications have very low yield of compliance in patients). Methotrexate is an effective agent, especially in patients with associated psoriatic arthritis, as it may halt or slow the degenerative changes. Liver toxicity from long-term use restricts its use to limited treatment cycles and patients with widespread disease not responsive to less aggressive modalities.
The synthetic retinoid, acitretin, has been shown to be effective in some patients with severe psoriasis, but it is a potent teratogen, thus limiting its use in women with childbearing potential. In addition, it has been shown as a superior treatment in pustular variants of psoriasis, but it is less successful in treating chronic plaque psoriasis.
PUVA (psoralen and ultraviolet A radiation) is a very effective treatment modality for psoriasis, but it has virtually no effect on psoriatic arthritis. Only approximately 5% of patients are unresponsive to this regimen, and as a rule they are patients who have prominent skin lesions on the face and hands (as these areas are normally photoexposed and presence of the lesions testifies to their resistance to ultraviolet radiation in daily sunlight). Topical steroids are commonly used for the skin lesions of psoriasis, but they have no effect on joint disease. Widespread use would also result in systemic absorption and systemic side effects.
Gonococcal arthritis: The presentation of asymmetric migratory polyarthralgia followed by monoarticular arthritis in a sexually active patient with a characteristic skin rash suggests gonococcal arthritis. The migratory polyarthralgia typically involves wrist, ankle, fingers, ankles or toes, asymmetrically as described by this patient. This occurs a week prior to the onset of mono articular arthritis. The involved joint shows signs of purulent inflammation such as erythema, tenderness, and low range of motion. The skin lesion consists of multiple necrotic pustules over palm and soles. All these symptoms and signs promptly resolve with administration of ceftriaxone, intravenously at first and later followed by oral administration. Patients of purulent gonococcal arthritis are usually afebrile.
Disseminated gonococcal infection is a syndrome of rash, tenosynovitis and polyarthralgia. Rash is pustular or vesiculopustular and is usually present on trunk and extensor surfaces of the distal extremities and it may become hemorrhagic. Tenosynovitis of multiple tendons at wrist, hands and ankles may occur. Arthralgia is migratory. Skin and joint manifestation are thought to be immune-mediated caused by immune-complex formation from gonococcemia. Women are at more likely to develop disseminated gonococcal infection especially during menses and pregnancy.
Lyme arthritis: occurs as a result of untreated Lyme infection. It is caused by Borrelia burgdorferi, a spirochete carried by the deer ticks. It travels through the blood stream into the various areas of the body, including the joints. The early symptoms are mild and are easily overlooked. Arthritis is the hallmark of late disease. It tends to involve the knee in most patients. Warmth, swelling from effusion and restriction of movements differentiate it from arthralgias, which occur early in the disease process. In this vignette, the patient has a history typical of intermittent inflammatory arthritis. The first episode occurs within six months of having erythema migrans. The episodes gradually disappear in about ten years, if left untreated. In the United States, Lyme disease is prevalent in Connecticut, Rhode Island, New York, Pennsylvania, New Jersey, Delaware, Maryland and Wisconsin.
Septic arthritis: presents with sudden onset of acute monoarticular arthritis in a previously damaged joint, associated with chills and fever. The joint involved will be tender and erythematous with a large effusion. It is most commonly caused by Staphylococcus aureus. It is common in intravenous drug abusers and immunocompromised patients.
Reactive arthritis: occurs two to four weeks after genitourinary or gastrointestinal infection. The onset is acute, with malaise and fever. The joints are symmetrically involved.
Parvovirus arthritis: Joint involvement in Parvovirus infection is symmetrical. Hands, wrists, knees and feet are the most frequently involved joints. Rash may or may not be present. Patient may have arthralgias or arthritis. Joint involvement most frequently occurs in adult female. Detection of Anti-B19 IgM antibodies is the diagnostic study of choice. These antibodies develop within 10-15 days of infection and they start decreasing after one or two months but may sometimes persist for 6 months. Persistence of IgM antibodies indicates chronic infection.
Whipple’s disease: is caused by bacteria Tropheryma whippelii and presents with arthralgias, abdominal pain, diarrhea and weight loss. Other organs like central nervous system, heart and skin may be involved. Patient may also have fever, chills and hyperpigmentation. Joint involvement is most frequently in the form of migratory polyarthralgias. Small intestinal biopsy is diagnostic test of choice and shows villous atrophy and Periodic Acid-Schiff positive deposits in lamina propria and these biopsy findings are pathognomonic of Whipple’s disease.
Inflammatory bowel disease in addition to producing intestinal disease and causing abdominal pain and diarrhea also has diverse extra-intestinal manifestations of peripheral arthritis, sacroiliitis, erythema nodosum, aphthous ulcers, pyoderma gangrenosum, and eye disease. P-ANCA is present in 60-80% of patients of Ulcerative colitis and 10-25% cases of Crohn' s disease.
Erythema nodosum (EN): It is characterized by pink to reddish painful, subcutaneous, nodules that usually develop in a pretibial location. EN occurs most often in women ages 15-40 years. Patients typically present with painful erythematous nodules developed on the anterior surfaces of both legs. They evolve into bruise-like lesions that resolve without scarring over a 2-6week period. Histologically, this is a panniculitis involving inflammation of septa in the subcutaneous fat tissue. EN is commonly associated with recent streptococcal infections. There are numerous other infections, which could cause EN. In Europe and North America, sarcoidosis constitutes a dominant cause of EN while tuberculosis (TB) remains an important underlying condition in other parts of the world. Thus, the initial work-up in excluding these diseases include antistreptolysin O (ASO) titer, a tuberculin skin test, and a chest radiograph.
EN is also associated with inflammatory bowel disease however EN develops around the time of gastrointestinal flares, in contrast to pyoderma gangrenosum, which commonly runs a course independent of gastrointestinal disease activity.
Sarcoidosis often presents with EN as an initial symptom. Chest x-ray in these patients may typically reveal bilateral hilar lymphadenopathy.
Reflex sympathetic dystrophy: is a syndrome of pain and swelling associated with vasomotor instability. Any extremity may be involved but is more common in the hand. Usually there is associated movement restriction in the shoulder. Direct injury, neck and shoulder injuries, or myocardial infarction can cause this condition. Examination reveals a discolored limb with atrophic changes. X-ray reveals osteopenia, bone scan shows increased uptake. Physical therapy is done to restore function. Prednisone is indicated in resistant cases. Stellate ganglion block is also very effective.
Anserine bursitis: Patients may reveal history of trauma. On examination, there is a well-defined area of tenderness over the medial tibial plateau just below the joint line. Valgus stress test does not aggravate the pain. X-ray of the tibia is normal.
Prepatellar bursitis presents with pain and swelling over the anterior aspect of knee. Examination shows cystic swelling over the patella with variable signs of inflammation.
Greater trochanteric bursitis is a common condition. Overlying the greater trochanter of the femur is a bursal sac that may become inflamed and painful. The bursa when palpated is often tender and will reproduce the patient’s symptoms. Antiinflammatory medication, injections, and if needed, surgical excision of the bursa are the mainstays of treatment.
Stress fractures: have been described for nearly every bone in the body. Stress fractures occur more frequently in athletes who suddenly increase their training load or have underlying osteopenia. A female athlete with a stress fracture should always be asked about her menstrual cycle and dietary habits. Stress fractures of the tarsal navicular bone are notorious for their slow healing rate because of the naviculars’ relatively poor blood supply. This patient should be seen by an orthopedic surgeon for further care; a bone scan or MRI would be a reasonable diagnostic study to confirm the diagnosis.
Metastatic bone lesions: is the most common malignant tumor of the skeletal system. Age greater than 50 years, pain, unexplained weight loss, and a history of cancer all should raise concern of metastatic disease. Prostate, breast, renal, lung, thyroid, and gastrointestinal malignancies tend to metastasize to the vertebral bodies, whereas certain primary tumors of bone are more likely to occur in the posterior elements of the spine (osteoid osteoma, osteoblastoma, and aneurysmal bone cysts). Technetium 99 scinti scanning is the most effective method to assess bone metastasis.
Spinal cord tumor: presents with leg weakness, paresthesias, bowel, and bladder dysfunction in patients with history of cancer. Compression of cord by the tumor leads to spasticity, hyperreflexia, and loss of temperature, position, and vibration sense.
Lumbar spinal stenosis: Pain of neurogenic claudication radiates back to the buttocks, upper or lower legs; it becomes better with sitting or leaning forward due to flexion of the lumbar spine or hips and gets worsened by extension of the lumbar spine. Wide gait and positive Romberg sign are specific but not sensitive for lumbar spinal stenosis.
The cauda equina syndrome: occurs as a result of compression of lumbosacral nerve roots by infection or tumor. Usual presentation is with urinary retention or overflow incontinence. Important physical findings include saddle anesthesia around the anus or perineum and decreased tone of anal sphincter.
Leriche syndrome: occurs as a result of atherosclerotic vascular disease and is characterized by impotence and intermittent claudication. This patient does have risk factors for intermittent claudication but his pain is different from intermittent claudication that does not occur at rest or standing and occurs only after walking and gets relieved by rest. Physical findings of patient with intermittent claudication may show absent or diminished pulses below the level of stenosis, low temperature of the involved extremity, bruit, impaired wound healing, increased venous filling time, shiny skin, hair loss, and atrophy of skin.
Disc herniation: presents as low back pain radiating down the buttock and below the knee. The pain is caused due to impinged nerve. A positive straight leg suggests nerve root irritation due to impingement. The crossed straight leg test is very specific for disc herniation.
Lumbar spinal stenosis: is associated with aging, especially after 60 years age. Narrowing of the spinal canal results from encroaching osteophytes at the facet joints, hypertrophy of the ligamentum flavum and protrusion of intervertebral disks. In some patients gait disturbance is so prominent that they complain of having “spaghetti legs” or walking “like a drunken sailor”. The preservation of pedal pulses helps distinguish from vascular claudication. The diagnosis of spinal stenosis in a patient with symptoms is best confirmed by using MRI of spine. X-ray will not be useful for the diagnosis of spinal stenosis.
Avascular necrosis of the femoral head: It represents an ischemic necrosis of the bone with eventual collapse of the periarticular bone and cartilage. Ischemic bone necrosis is due to compromised blood flow to the femoral head that can be traumatic or non-traumatic. The well-known causes of non-traumatic avascular (aseptic) necrosis are chronic corticosteroid therapy, alcoholism, and hemoglobinopathies. The clinical scenario described is typical (progressive hip pain without restriction of motion range and normal radiograph on early stages). A high index of suspicion is required, because early diagnosis is desirable whether nonoperative or operative treatment is considered; therefore, knowing the predisposing factors is important. MRI and scintigraphy are used to confirm the diagnosis.
A compression fracture of the vertebrae: is a common complication of advanced osteoporosis. It usually manifests as acute back pain without an obvious preceding trauma in a predisposed patient. Neurologic examination will be normal.
Note: Absent ankle reflex can be seen in elderly patients as part of the normal aging process.
Neuropathies: Ulnar neuropathy at the elbow is the second most common compression neuropathy. Compression of the nerve at the epicondylar groove is a common site of compression. A positive Tinel's sign (paresthesias or dysesthesias when tapping over the affected nerve), paresthesias of digits 4 and 5, and weakness in finger and wrist flexion may be present in severe cases.
Median nerve entrapment commonly occurs at the carpal tunnel. It presents as painful paresthesias of digits 1 to 4, especially while sleeping (patients may find relief by waking and shaking their hands) or performing activities that result in wrist strain, such as driving or working at a keyboard.
Radial neuropathies result in wrist, finger, and forearm extension weakness. The radial nerve is often entrapped at the spiral groove, which is midway down the humerus.
A brachial plexopathy will involve components of multiple nerves, depending on where the lesion is. A history of trauma in an adult (arm weakness and rotation after a motorcycle accident) or in a child (traumatic birth, or being pulled up by the arms) is a more common presentation of a brachial plexopathy.
Radiculopathy is rarely spontaneous in young, otherwise healthy patients. It will usually present with severe radiating pain, sensory loss, and weakness in the muscles of the myotome innervated by the specific nerve root. Cervical trauma may have occurred.
Carpal tunnel syndrome occurs following entrapment of the median nerve within the carpal tunnel. These patients usually complain of numbness, pain, and paresthesias along the median nerve distribution. There is diminished two-point discrimination over the affected areas. They have weakness in the affected limb and frequently drop things. It is commonly seen in individuals with history of repetitive hand movements. It is seen in association with rheumatoid arthritis, myxedema, amyloidosis, sarcoidosis, leukemia, and hyperparathyroidism. Nerve conduction studies are more reliable in diagnosing carpal tunnel syndrome. Sensory conduction delay is usually more prominent than motor weakness.
Phalen’s sign – flexion of both wrists to 90 degrees with the dorsal aspect of hands held in opposition for 60 seconds leads to pain or paresthesia along the median nerve. This is a positive Phalen’s sign. This test has a good specificity but lower sensitivity. Carpal compression test is performed by, applying pressure over the carpal tunnel. This reproduces the patient’s symptoms. This test is more sensitive than Tinel’s and Phalen’s signs but not diagnostic. Tinel’s sign is elicited by tapping over the median nerve. This causes a tingling sensation along the median nerve distribution. It has very low sensitivity and specificity.
Preservation of muscle mass and sensory perception indicates an early, mild process. Splints provide relief to most patients at this stage. Steroid injections are the next level of therapy. They can help in more advanced disease. They should not be the first line of therapy.
Radial tunnel syndrome: can be confused with lateral epicondylitis as both the conditions can coexist. It is a compression neuropathy of radial nerve in radial tunnel characterized by tenderness over mobile muscle mass distal to the radial head. Also, the pain is reproduced by simultaneously extending the wrist and fingers while the long finger is passively flexed by the examiner and also by resisted forearm supination.
Dupuytren’s contracture: occurs in patients over the age of 50, commonly with history of diabetes mellitus, chronic alcoholism, epilepsy, or tuberculosis. It is also associated with Peyronie’s disease, retroperitoneal fibrosis, and Reidel’s thyroiditis. It manifests as nodular thickening of the third and the fourth fingers with inability to extend completely. No functional loss occurs. Steroid injections are helpful in some patients. In others surgical ligation may be used.
De Quervain’s disease: is characterized by tendonitis of abductor pollicis longus and extensor pollicis brevis as they pass beneath the retinacular pulley. It was originally described in washerwomen in whom these tendons were chronically irritated by wringing the clothes. However, in present times it is most commonly seen in post partum females due to repetitive lifting of the infant. Tenderness is noted over the radial side of wrist and first dorsal compartment. Clinical diagnosis can be made by positive Finkelstein maneuver (passive stretching of the thumb tendons over the radial styloid in thumb flexion aggravates pain). Treatment is best with local injection of long-acting steroids.
Blue toe syndrome: It is characterized by intact pulses, painful cyanotic toe along with features of ischemic lesions in distal limbs, such as livedo reticularis. The problem can occur spontaneously, may occur secondary to surgical manipulations. Labs are significant for increased ESR, hypocomplementemia and eosinophilia. Features of renal involvement may occur. Livedo reticularis refers to mottled discoloration of the extremities.
Erythromelalgia: is a paroxysmal disorder of peripheral blood vessel dilation with bilateral burning pain on palms and soles. It then progresses to the entire extremity. The area usually becomes red and warm.
Reflex sympathetic dystrophy: refers to burning pain in an area with previous history of trauma. Symptoms refer to vasomotor instability with changes in temperature, color, and texture of skin. This patient had no such symptoms.
Ganglion cyst: presents as small nodule that is mobile and tender to touch and is usually located over the metacarpophalangeal joint.
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| drtanvir
Forum Junior
Topics: 14
Posts: 41
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An interdigital neuroma (Morton's neuroma): is a compression of the interdigital nerve at the area between the metatarsal heads and under the deep transverse metatarsal ligament, usually in the third webspace. Usually it represents a perineural fibrosis rather than an actual neuroma, The female to male ratio if approximately 4:1 and the average age of the patient is 55 years. The condition usually is unilateral and is aggravated by tight shoe wear. The most common symptom is plantar pain that is increased by weight bearing with a burning sensation. Pain usually is relieved by rest and removing the shoes. Conservative care includes wearing a shoe with an unconstricting toe box, a metatarsal pad proximal to the involved webspace, steroid injections, and antiinflammatory medications. If conservative measures fail, surgery usually is recommended to remove the nerve.
Lateral epicondylitis (tennis elbow): Tennis elbow is epicondylitis about the origin of the extensor muscle from lateral epicondyle of the humerus. It is often seen in tennis players in their fourth or fifth decade and is due to repeated forceful wrist extension. It is characterized by point tenderness over the lateral epicondyle of humerus and exacerbation of pain by extension of wrist against resistance.
Epicondylitis about the origin of flexors of forearm is also called golfer’s elbow or medial epicondylitis and is characterized by point tenderness over the medial epicondyle and pain, which is exacerbated by flexion at wrist against resistance.
Rotator cuff tear: presents with shoulder pain aggravated by movements like pushing, pulling and positioning the arm above the shoulder as well as weakness of shoulder resulting in functional impairment. Examination may show limitation of mid arc abduction or external rotation or both which does not reverse with lidocaine injection thus differentiating the rotator cuff tear from rotator cuff tendinitis.
Definitive diagnosis of rotator cuff tear requires MRI or arthrography. MRI should be performed when rotator cuff tear is suspected on clinical grounds and patient has no other serious medical problem that will make the corrective surgery difficult. The above patient does not have any serious medical problem and his symptoms are incapacitating and he is very likely to be benefited from surgery.
Rotator cuff tendinitis can be distinguished from rotator cuff tear by injecting lidocaine that will result in improvement in range of motion in cases of rotator cuff tendinitis but no effect in range of motion in cases of rotator cuff tear.
Frozen shoulder: there is limited range of motion due to stiffness of glenohumeral joint. Chronic pain may be present but the most prominent symptom is joint stiffness. On examination, range of motion is restricted both on active and passive movement in all cardinal movements. This is a result of pericapsulitis. Majority of the cases are idiopathic. Arthroscopy establishes the diagnosis by showing decreased joint space volume, and loss of normal axillary pouch. The treatment involves NSAIDs, corticosteroids injection into the joint space, and physical therapy.
Subacromial bursitis: Injury to rotator cuff is a common cause of shoulder pain and disability in athletes. Prolonged, repetitive overhead activity as in tennis, swimming, pitching or golf can compromise the space between the humeral head and coracoacromial arch leading to impingement syndrome. Subacromial bursitis results from impingement syndrome. It refers to inflammation of the subacromial bursa. Rotator cuff tendon tear and supraspinatus tendonitis can also occur as a part of impeachment syndrome. Subacromial bursitis is characterized by shoulder pain, which is absent at rest but present on overhead activity. Range of active shoulder movements are limited by pain. Neer’s impingement sign (pain on passive internal rotation and forward flexion at shoulder) is present. No atrophy of shoulder muscle is present. Ultrasonogram or MRI generally confirms the diagnosis. Treatment is conservative with NSAIDs, physical therapy and activity modification.
Cellulites:which is a diffuse infection of deep layers of skin. Presence of fever, inflammatory signs, no crepitus or bullae and signs of overlying skin necrosis all suggest of cellulitis. Toe web tinea pedis is one of the most common portals of entry for the microorganisms, causing cellulitis.
Cellulitis of calf is difficult to differentiate from deep venous thrombosis. However presence of high-grade fever, lymphangitis, absence of any risk factor such as orthopedic surgery, or prolonged immobilization make deep venous thrombosis unlikely.
Necrotizing fasciitis is a deep-seated cellulitis. It should be suspected in a patient who has evidence of overlying skin necrosis, bullae, with anesthesia due to destruction of nerves, crepitus due to gas producing organism, and fever.
Patellofemoral syndrome: a self-limited condition, occurs more frequently in females under the age of 45 and presents with anterior knee pain. Pain is aggravated by flexion. Examination shows retropatellar tenderness and crepitation i.e. pressure on the patella causes severe pain.
Thoracic outlet syndrome: refers to compression of the neurovascular structures supplying the upper extremity. It may be caused due to compression by scalene muscles, cervical ribs or congenital fibro muscular band. Motor vehicle accidents, playing musical instruments and chronic illnesses are other predisposing factors. Pain usually occurs from the point of compression to the axilla, shoulder, arm, forearm, and hand. Paresthesias are usually aggravated at night. Motor abnormalities include weakness and muscle atrophy. Vascular involvement presents as pallor, pulselessness, and coolness of the affected side; venous obstruction is marked by edema and cyanosis. An elevated arm stress test (EAST) is used for screening purposes. Chest x-ray, MRI, and angiography help in diagnosing the cause of compression. Conduction velocity studies identify the site of compression. Overhead pulley exercises, shoulder bracing and other postural changes help to relieve compression.
Polymyelgia Rehumetica: characteristic findings are age of patient greater than 50, morning stiffness for 30 minutes or more and present for more than a months and it involves shoulder and hips. ESR is greater than 40 mm/hour. Most commonly pain and stiffness occur in a shoulder/trapezius, hip/girdle distribution. The diagnosis is supported by an elevated ESR or C-reactive protein (CRP).
The treatment of choice for polymyalgia rheumatica is with low-dose prednisolone. NSAIDs may be needed to treat mild discomfort when glucocorticoids are being tapered. Polymyalgia rheumatica may be present alone or in 40-50% cases, it is associated with giant cell arteritis. High-dose prednisolone therapy is used when giant cell arteritis is present. Temporal artery biopsy is required to confirm the diagnosis of giant cell arteritis. Features of giant cell arteritis include headache, jaw-claudication, transient visual loss and palpable tender arteries of scalp, neck or extremities.
Fibromyalgia: Presence of diffuse musculoskeletal pain, multiple tender points with no joint swelling/pain, usually makes the diagnosis. Initial evaluation should include a Complete blood count, ESR, Thyroid function tests (TFTs) and enzymes (CK). Hypothyroidism typically can present like this. It is far more common in females and usually presents between 35-55.
Hypothyroidism may be very difficult to differentiate from fibromyalgia, as it is often associated with generalized aches, fatigue, and disturbed sleep. So, TFTs should be routinely obtained during the initial evaluation of patients with suspected fibromyalgia.
Fibromyalgia is not an inflammatory condition and therefore NSAIDs such as naproxen or glucocorticoids have not been shown to be very effective in its treatment.
Tricyclic antidepressants like Amitriptyline have been shown to be effective in the treatment of fibromyalgia and they are usually given in low doses at nighttime. To relieve the pain, non-narcotic analgesics like acetaminophen can be given during the daytime. The only other drug other than tricyclic antidepressants studied extensively in fibromyalgia is cyclobenzaprine. So either amitriptyline or cyclobenzaprine are the initial drugs of choice. Narcotic analgesics like Oxycodone need to be avoided. Selective serotonin reuptake inhibitors (SSRIs) are added when disease is refractory to the above medicine. When patient feels better with the pharmacological treatment exercise program is initiated. The other treatment that can be tried in refractory cases are trigger point injections.
Chronic fatigue syndrome: although resembles Fibromyalgia, patients mostly complain of extreme fatigue and not body aches. No trigger points can be demonstrated; symptoms should be present for at least 6 months to make the diagnosis of chronic fatigue syndrome.
Gout: Gout results from deposition of monosodium urate crystals into the joint space. Nearly all patients with gout are hyperuricemic, but only 5% of patients with hyperuricemia have gout. Gout is an exquisitely painful monarthritis, but may be accompanied by polyarticular involvement. There may be fever. Most attacks subside after days or weeks. Gout usually occurs in the big toe, but in patients with untreated hyperuricemia, nodular gout may occur in the ear, ulnar surface of forearm, achilles tendon, and fingers. The sudden onset of pain and swelling in the metatarsophalangeal joint is suggestive of podagra, the common site of involvement in gout. This is more common in patients with myeloproliferative disorders, chronic renal disease, psoriasis, hemoglobinopathies and using medications such as thiazide, niacin etc. This is a metabolic disorder associated with hyperuricemia either due to over production or under secretion of uric acid. The demonstration of needle shaped urate crystals in the synovial fluid is diagnostic of gout; hence a joint fluid aspirate should be done. You should never leave a tender inflamed joint without aspiration. Tophi are nodular deposits of monosodium urate monohydrate crystals with associated foreign body reaction. Serum uric acid levels tend to be normal in some patients with acute gout, hence not very helpful in diagnosis.
In patients with frequent attacks of acute gouty arthritis not controlled by colchicine, a 24-hour uric acid levels in urine is determined. This evaluates whether hyperuricemia is due to over production or under secretion of uric acid. A value of less than 800 mg/day suggests under secretion. A value of more than 800 mg/day suggests over production. Hence accordingly a uricosuric agent or a xanthine oxidase inhibitor is added to the patient’s medication. The goal is to maintain serum uric acid levels to less than 6 mg/dL.
Pseudogout: is characterized by acute inflammatory changes of the large joints, most commonly the knee joint. It is associated with fever, chills, and leukocytosis. Synovial fluid analysis may show a leukocyte count as high as 100,000/cmm. Do not confuse with septic arthritis. Gram stain is usually negative in pseudogout. Episodes may be precipitated by surgery or trauma and can last up to 10 days if not treated. The diagnosis is confirmed by the finding of positively birefringent calcium pyrophosphate crystals and articular calcification.
Hemochromatosis is the only condition that has been found to be clearly associated (50%) with pseudogout. Pseudogout also occurs more frequently in patients with transfusion hemosiderosis. Other disorders associated with pseudogout include hyperparathyroidism, hypomagnesemia, hypophosphatemia and hypothyroidism.
Polymyositis: is an inflammatory muscle disease of unknown etiology. It presents as proximal weakness characterized by difficulty ascending and descending stairs, combing hair, kneeling down etc. There are no skin rash or scaly patches as seen in dermatomyositis. Though some patients present with muscle pain and tenderness, it is not very typical in majority. These patients have dysphagia due to involvement of the striated muscles of the upper pharynx. It shows endomysial infiltration of the inflammatory infiltrate. These conditions may occur alone or in association with a variety of neoplasms such as: breast, ovary, lung, prostate, or colon cancer. Suspected patients should have measurement of serum creatine kinase and aldolase levels. Muscle biopsy is the best diagnostic study for polymyositis.
Generally, polymyositis and dermatomyositis respond well to prednisone therapy. Cases associated with neoplasia respond less well, but may improve after removal of the associated tumor. In case the patient fails to improve on or cannot tolerate the side effects of corticosteroid therapy, immunosuppressive agents such as methotrexate may be effective.
Systemic sclerosis: is a chronic autoimmune disorder, which is characterized by fibrosis of skin and internal organs. There are two forms of systemic sclerosis: 1) limited, which is more common and 2) diffuse which occurs only in 15-20% of cases. The most characteristic findings of this disease are prominent vasomotor changes, fibrosis and subsequent atrophy of the skin, subcutaneous tissue, muscles, and internal organs, and immunologic changes. It is caused by excessive collagen deposition in the skin and internal organs. The affected organs and systems include the skin, heart, lungs, gastrointestinal tract, kidneys, muscle, joints, and nervous system. Raynaud’s phenomenon is a common finding, with fingers going through blanching, cyanosis, and erythema, accompanied by substantial pain. Subsequently, small, painful trophic ulcerations with or without subcutaneous calcification develop on the fingertips. Extensive disease may cause gangrene of the distal digits. Renal failure and pulmonary fibrosis are the most common causes of death. The characteristic antibody detected in patients with systemic sclerosis and interstitial lung fibrosis is antibodies against DNA topoisomerase I (Scl 70). Antibodies to U3-RNP are associated with systemic sclerosis and particularly correlate with progression to pulmonary hypertension.
CREST syndrome: is a form of scleroderma that is localized. In this form hardening of the skin is limited to the face and hands whereas in the diffuse form it extends to involve the trunk and proximal extremities. CREST is an acronym for the cardinal features of this syndrome Calcinosis, Raynaud’ s phenomena, Esophageal dysfunction, Sclerodactyly, and Telangiectasias. Calcinosis presents as nodular swellings, which may be symptomatic or asymptomatic. If symptomatic it presents with pain and tenderness, sometimes it may ulcerate and exude a white chalky fluid. X-ray of the involved area shows calcifications. CREST syndrome has a much better prognosis than diffuse scleroderma. These patients do not develop interstitial lung disease or renal failure as in diffuse form.
Eosinophilic fasciitis: also presents with diffuse skin changes similar to systemic sclerosis. The inflammation is limited to the fascia and does not involve the epidermis or the dermis. These patients do not have Raynaud’ s phenomena.
Eosinophilia myalgia syndrome: is seen in patients on tryptophan used for insomnia and pre menstrual syndrome. It is an essential amino acid and is currently banned by the food and drug administration. These patients develop diffuse myalgia and skin changes in the form of hives, generalized swelling and induration of the limbs.
Staphylococcal toxic shock syndrome: is caused by exotoxin and is characterized by fever, macular erythroderma of palms and soles, vomiting, diarrhea and hypotension. Use of highly adsorbent tampon is an important risk factor. Streptococcal toxic shock syndrome presents in a similar manner and follows invasive streptococcal infections.
Mixed Connective Tissue Disease (MCTD): Diagnosis of Mixed Connective Tissue Disease (MCTD) is made when serology shows positive anti-RNP antibodies and clinical features of SLE, polymyositis and systemic sclerosis are present simultaneously. These features include swollen hands, synovitis, myositis, acrosclerosis, and Raynaud’s phenomenon. If three of these features are present, along with positive anti-RNP, diagnosis of MCTD is established. This occurs in a much younger population. It later evolves into any of the rheumatic disorders.
Dermatomyositis: an inflammatory myositis characterized by edema, dermatitis, and muscular inflammation with degeneration. With or without skin lesions, weakness of proximal muscle groups is characteristic. The cutaneous signs of dermatomyositis may precede or follow the development of myositis by weeks- years. Periorbital edema and a purple-red discoloration of the upper eyelids, sometimes associated with scaling (heliotrope erythema) are the most common skin manifestations. Erythema and scaling may be particularly prominent over the elbows, knees, and the interphalangeal joints. A reddish-purple, scaling eruption occurs over the metacarpal-phalangeal joints, elbows, and knees (Gottron's sign) with flat-topped, violaceous papules pathognomonic of dermatomyositis over the metacarpal-phalangeal joints (Gottron's papules). Hyperkeratosis, scaling, fissuring, and hyperpigmentation over the fingertips, sides of the fingers, and palms is referred to as mechanic’s hands, and has been associated with antisynthetase antibodies in 70% of patients. Photosensitivity is common, and most skin lesions are present in sun-exposed areas. Pruritus is variable, from none to devastating. Intermittent fever, malaise, anorexia, arthralgia, and marked weight loss are present at this stage. In severe cases, early and extensive muscular weakness occurs with acute swelling and pain. It most frequently involves the shoulder girdle and pelvic region in a symmetric way. Patients may not be able to raise their arms to undress or comb their hair, or they may have difficulty rising from a sitting position, squatting, or climbing stairs. Other involved muscle groups may present with difficulty swallowing, talking, or breathing. In the terminal phase of the disease, cardiac involvement with failure may be present. The following criteria are used to define dermatomyositis/polymyositis:
Suspected patients should have measurement of serum creatine kinase and aldolase levels. Specific diagnosis is usually made by muscle biopsy. These patients should have a complete metastatic workup to rule out malignancy. It includes PAP smear, mammogram, CT chest / abdomen / pelvis, and colonoscopy.
Prednisone is the mainstay of treatment, along with steroid-sparing immunosuppressive agents such as methotrexate, azathioprine, and cyclosporine.
SLE: a severe, life-threatening autoimmune connective tissue disease. Skin involvement occurs in 80% of cases and is likely to be very helpful in making the correct diagnosis. The features includes:
1.Systemic symptoms: Fatigue, fever, and weight loss.
2.Cutaneomucous symptoms: Malar rash, discoid rash, alopecia, oral or nasopharyngeal ulcers, and photosensitivity. The lupus band test is direct immunofluorescence of non–sun-exposed skin. A positive lupus band test correlates with aggressive course of systemic disease in patients with systemic lupus erythematosus. 3.Serositis: pericarditis, pleuritis
4.Nephritis: which can be
-Mesangial, which is characterized by focal and segmental glomerular involvement with an increase in mesangial cells;
-Focal proliferative form;
-Diffuse proliferative form, which is characterized by a "wire loop" pattern resulting from immune complex deposition and subsequent thickening of the glomerular basement membranes. It can result in irreversible changes and chronic renal failure
-Membranous form, which is similar to primary membranous glomerulonephritis.
5.Neurologic symptoms: Depression, psychosis, seizures, and neuropathy.
6.Non-erosive arthritis: Arthritis and arthralgias of SLE are migratory and asymmetrical.
7.Hematologic disease: Hemolytic anemia; leukopenia; lymphopenia; thrombocytopenia.
The serologic pattern of the disease is characterized by the presence of positive antinuclear antibodies; anti-dsDNA antibodies; anti-Sm antibodies;
SLE in some cases presents with renal involvement. Although SLE can cause several types of immune-mediated tissue damage and at the same time causes several well defined kidney diseases, it is believed that the main mechanism of kidney damage in SLE patients is immune complex-mediated. Immune complexes circulate in the blood and are deposited in renal glomeruli causing their damage via several mechanisms. One of these mechanisms includes complement activation. That’s why serum C3 level is decreased in immune complex-mediated kidney diseases such as SLE and poststreptococcal glomerulonephritis. When kidney is involved, a SLE renal biopsy is needed, especially in those cases where renal involvement is less severe with mild proteinuria and hematuria or when presentation is of nephrotic syndrome with bland urinary sediment. Therapy is then directed by pattern of glomerular involvement. Renal involvement is very common in patients of systemic lupus erythematosus (SLE). Renal biopsy shows involvement of kidneys in more than 90% patients of SLE. Abnormal urinalysis is present in more than 50 percent patients of SLE. Most common type of renal injury in patients of SLE is immune mediated glomerular injury that can be divided into six sub-groups. Type I is normal. Remaining five are as follows: Mesangial, focal proliferative, diffuse proliferative, membranous and sclerosing.
Mesangial is the earliest and the least severe form and is present in 10-20 percent of cases. Anti-DsDNA titers are not very high.
Focal proliferative is a severe form than Mesangial form and is present in 10-20 percent of cases. Urinalysis shows proteinuria and hematuria and serum creatinine is elevated. Histopathology shows proliferative changes with some areas of necrosis. Less than 50 percent of glomeruli are affected.
Diffuse proliferative is the most frequent pattern of renal involvement and unfortunately is the severest form. Hematuria, proteinuria, renal insufficiency, hypertension, marked hypocomplementemia and marked elevation of anti-DsDNA are all present. Histopathology shows changes that are similar but more severe than in focal proliferative type. More than 50 percent of glomeruli are affected. It has the worst of all prognosis.
Membranous glomerulonephritis is present in 10-20% cases of SLE and usually presents with nephrotic syndrome. Renal function is preserved. Basement membrane is thickened and subepithelial deposits are present. It has better prognosis than diffuse proliferative type.
Sclerosing type represents healing of previous inflammatory damage and presents with renal insufficiency and normal urinary sediment. Immunosuppressive therapy is not effective in such patients as there is no active inflammation.
Oral contraceptive pills use in SLE is considered safe if the patient is in remission. In patients with active nephritis, oral contraceptive pills shouldn’t be used as it can aggravate renal disease. Barriers method is used instead.
Anti phospholipid antibody syndrome: is characterized by recurrent arterial or venous thrombosis or recurrent fetal losses in the presence of anti phospholipid antibodies. Anti phospholipid antibodies are of three types. The first is responsible for false-positive syphilis serology. The second is lupus anticoagulant, which falsely elevates APTT. The third is anticardiolipin antibody. Antiphospholipid antibody syndrome is either primary or may be associated with other autoimmune disorders like SLE.
Sarcoidosis: Sarcoidosis is a systemic disease characterized by granulomatous inflammation of the lung. It is more common in African-American women in their third and fourth decades. It presents with cough, dyspnea, non-specific chest pain and skin lesions characteristic of erythema nodosum. It should be suspected in patients with bilateral ankle arthritis and bilateral hilar adenopathy on chest-x ray.
Sarcoidosis is basically two types: Acute and chronic. Lofgren’s syndrome is an acute form of sarcoidosis predominantly seen in Scandinavian, Irish, and Puerto Rican woman. The characteristic presentation consists of triad of arthritis (of ankles, knees, wrists or elbows), erythema nodosum and bilateral hilar adenopathy. Lung involvement is very rare in this acute form. Laboratory studies suggest inflammatory disease with elevated ESR, false positive rheumatoid factor, and antinuclear antibodies.
Heerfordt-Waldenstrom syndrome is another rare from of acute sarcoidosis consists of fever, parotid enlargement, anterior uveitis, and facial nerve palsy.
Chronic sarcoidosis has an insidious onset that develops over months. It mainly presents with respiratory complaints without constitutional symptoms. Very few patients have organs other than lung involved. It leads to permanent lung damage.
Systemic steroids are the drugs of choice for sarcoidosis and indicated in patients with disabling symptoms, organ dysfunction, organ derangements, and abnormal serum tests. Steroids effectively suppress the activated T helper-inducer cell processes occurring at the disease site. Asymptomatic sarcoidosis on the other hand does not require treatment.
Cyclosporine is mainly used in patients with extra thoracic sarcoidosis, not responding to systemic steroids.
Osteogenesis imperfecta—an autosomal dominant disease. The most common molecular defect is a mutation in one of the two genes encoding type I procollagen.
Type II procollagen gene mutation is found in patients with chondrodysplasia—an inherited skeletal disorders that cause dwarfism, ocular changes and cleft palate.
Type III procollagen gene mutation is found in patients with Ehlers-Danlos syndrome, that is characterized by hyperelasticity of the skin and hypermobile joints.
Paget’s disease: of the bone is characterized by excessive bone destruction and repair. It generally is asymptomatic and may be suspected by findings of elevated levels of alkaline phosphatase. The serum calcium is usually normal. Over a period of time 1-3% of individuals will develop osteosarcoma or sarcomatous changes. This will manifest itself as marked bone pain, new lytic changes and a sudden increase in alkaline phosphatase.
Wegener’s granulomatosis: is a granulomatous vasculitis-affecting upper and lower respiratory tracts as well as causing glomerulonephritis. Patient usually presents with hematuria, hemoptysis and features of upper respiratory tract or sinus involvement. Labs show elevated c-ANCA.
Churg Strauss syndrome: is a multisystem vasculitis disorder of unknown etiology that affects skin, kidney, nervous system, lungs, gastrointestinal tract and heart. There is a history of asthma followed by the development of fever and marked eosinophilia at which time asthma may become better. The disease is severe and requires treatment with glucocorticoids and sometimes with immunosuppressants.
Polyarteritis nodosa: involves medium sized arteries and most frequently organs include kidney, liver, gastrointestinal tract, skin, heart and peripheral nerves. It has been found to be associated with hepatitis B and C. Labs shows elevated P-ANCA.
Buerger’s Disease: It is an episodic, and segmental inflammatory process of small and medium sized arteries (thromboangiitis obliterans). It is characterized by occlusive disease of the arteries, migratory superficial thrombophlebitis, and Raynaud’s phenomenon. Usually one or two of these three conditions are present. Other important features are young age, males and history of smoking. Cessation of smoking is an important component of management.
Takayasu’s arteritis (Pulseless disease): is of unknown cause, and usually involves branches of the aortic arch. The symptoms caused by this disease are caused by cerebrovascular insufficiency, such as absent pulses in upper limb and neck bruit.
Giant cell arteritis: should always be suspected in a patient older than 50 years who presents with headache, visual problems and polymyalgia rheumatica. Such patients may also have weight loss, anemia and high ESR. Lab studies may shows elevated platelet counts and normocytic normochromic anemia. Therapy with high dose glucocorticoids is the treatment of choice and should be started immediately when diagnosis of giant cell arteritis associated with visual disturbances is strongly suspected. Blood samples can be drawn for ESR before the start of glucocorticoid therapy and temporal artery biopsy that is required to confirm the diagnosis needs to be done after starting steroid treatment. One should not wait for temporal artery biopsy before starting glucocorticoid therapy as any delay in the treatment may result in visual loss. Treatment with steroids does not affect the results of temporal artery biopsy. High dose steroid therapy is continued till the symptoms resolve and labs become normal.
Methotrexate is also effective in the treatment of giant cell arteritis but it is used only as a steroid-sparing agent and is not indicated at this time.
Thoracic aortic aneurysm, that is 16 times more common in patients of giant cell arteritis than the general population, is a serious complication and such patient need to have continuous monitoring. This aneurysm may dissect and prove to be fatal. The possible explanation for the development of aneurysm is disruption of collagen and elastin by chronic aortitis.
Cryoglobulinemia:Hepatitis-C virus infection account for at least 80% of the vasculitis cases associated with mixed cryoglobulinemia. Therefore all such patients should be tested for hepatitis C virus (HCV) infection by serum HCV antibody or HCV RNA. If these tests are negative, cryoprecipitate can be tested for HCV antibody or HCV RNA.
Only few cases (case reports) of mixed Cryoglobulinemia are due to infection with HBV virus and HIV virus, therefore testing for these viruses is not routinely indicated in patients of mixed cryoglobulinemia.
Cyclophosphamide toxicity: Cyclophosphamide produces many adverse effects, the most frequent of which include alopecia, sterility, amenorrhea, nausea, vomiting diarrhea, mucositis, stomatitis, acute hemorrhagic cystitis, bladder carcinoma, and blood dyscrasias. The proposed mechanism for acute hemorrhagic cystitis and bladder cancer is irritation by acrolein that is a metabolite of cyclophosphamide. Taking plenty of fluids, voiding frequently and avoiding the drug at night can prevent this side effect. Use of mesna is sometimes indicated for this purpose.
Hydroxychloroquine is the safest drug for systemic lupus erythematosus (SLE). However, rarely, it may cause serious eye disease including macular degeneration; so eye examinations at 6 months to 1 year intervals should be performed in all such patients who are taking this drug. Visual field defects, impaired color vision, bull’s eye pattern on funds and corneal whirls can all occur with its use.
The most common side effects of hydroxychloroquine are allergic skin reactions and nausea but they are not serious ones. The incidence of nausea can be reduced if it is taken after meals. Contraindications to its use include G6PD deficiency, porphyria cutanea tarda, liver failure and hepatic failure. It is also need to be avoided in pregnancy.
Though it may cause hepatic failure, deafness and blood dyscrasias they are very rare and can be monitored by liver function tests (LFTs), hearing examination and CBC with differential respectively. This monitoring is not recommended regularly.
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| drtanvir
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An interdigital neuroma (Morton's neuroma): is a compression of the interdigital nerve at the area between the metatarsal heads and under the deep transverse metatarsal ligament, usually in the third webspace. Usually it represents a perineural fibrosis rather than an actual neuroma, The female to male ratio if approximately 4:1 and the average age of the patient is 55 years. The condition usually is unilateral and is aggravated by tight shoe wear. The most common symptom is plantar pain that is increased by weight bearing with a burning sensation. Pain usually is relieved by rest and removing the shoes. Conservative care includes wearing a shoe with an unconstricting toe box, a metatarsal pad proximal to the involved webspace, steroid injections, and antiinflammatory medications. If conservative measures fail, surgery usually is recommended to remove the nerve.
Lateral epicondylitis (tennis elbow): Tennis elbow is epicondylitis about the origin of the extensor muscle from lateral epicondyle of the humerus. It is often seen in tennis players in their fourth or fifth decade and is due to repeated forceful wrist extension. It is characterized by point tenderness over the lateral epicondyle of humerus and exacerbation of pain by extension of wrist against resistance.
Epicondylitis about the origin of flexors of forearm is also called golfer’s elbow or medial epicondylitis and is characterized by point tenderness over the medial epicondyle and pain, which is exacerbated by flexion at wrist against resistance.
Rotator cuff tear: presents with shoulder pain aggravated by movements like pushing, pulling and positioning the arm above the shoulder as well as weakness of shoulder resulting in functional impairment. Examination may show limitation of mid arc abduction or external rotation or both which does not reverse with lidocaine injection thus differentiating the rotator cuff tear from rotator cuff tendinitis.
Definitive diagnosis of rotator cuff tear requires MRI or arthrography. MRI should be performed when rotator cuff tear is suspected on clinical grounds and patient has no other serious medical problem that will make the corrective surgery difficult. The above patient does not have any serious medical problem and his symptoms are incapacitating and he is very likely to be benefited from surgery.
Rotator cuff tendinitis can be distinguished from rotator cuff tear by injecting lidocaine that will result in improvement in range of motion in cases of rotator cuff tendinitis but no effect in range of motion in cases of rotator cuff tear.
Frozen shoulder: there is limited range of motion due to stiffness of glenohumeral joint. Chronic pain may be present but the most prominent symptom is joint stiffness. On examination, range of motion is restricted both on active and passive movement in all cardinal movements. This is a result of pericapsulitis. Majority of the cases are idiopathic. Arthroscopy establishes the diagnosis by showing decreased joint space volume, and loss of normal axillary pouch. The treatment involves NSAIDs, corticosteroids injection into the joint space, and physical therapy.
Subacromial bursitis: Injury to rotator cuff is a common cause of shoulder pain and disability in athletes. Prolonged, repetitive overhead activity as in tennis, swimming, pitching or golf can compromise the space between the humeral head and coracoacromial arch leading to impingement syndrome. Subacromial bursitis results from impingement syndrome. It refers to inflammation of the subacromial bursa. Rotator cuff tendon tear and supraspinatus tendonitis can also occur as a part of impeachment syndrome. Subacromial bursitis is characterized by shoulder pain, which is absent at rest but present on overhead activity. Range of active shoulder movements are limited by pain. Neer’s impingement sign (pain on passive internal rotation and forward flexion at shoulder) is present. No atrophy of shoulder muscle is present. Ultrasonogram or MRI generally confirms the diagnosis. Treatment is conservative with NSAIDs, physical therapy and activity modification.
Cellulites:which is a diffuse infection of deep layers of skin. Presence of fever, inflammatory signs, no crepitus or bullae and signs of overlying skin necrosis all suggest of cellulitis. Toe web tinea pedis is one of the most common portals of entry for the microorganisms, causing cellulitis.
Cellulitis of calf is difficult to differentiate from deep venous thrombosis. However presence of high-grade fever, lymphangitis, absence of any risk factor such as orthopedic surgery, or prolonged immobilization make deep venous thrombosis unlikely.
Necrotizing fasciitis is a deep-seated cellulitis. It should be suspected in a patient who has evidence of overlying skin necrosis, bullae, with anesthesia due to destruction of nerves, crepitus due to gas producing organism, and fever.
Patellofemoral syndrome: a self-limited condition, occurs more frequently in females under the age of 45 and presents with anterior knee pain. Pain is aggravated by flexion. Examination shows retropatellar tenderness and crepitation i.e. pressure on the patella causes severe pain.
Thoracic outlet syndrome: refers to compression of the neurovascular structures supplying the upper extremity. It may be caused due to compression by scalene muscles, cervical ribs or congenital fibro muscular band. Motor vehicle accidents, playing musical instruments and chronic illnesses are other predisposing factors. Pain usually occurs from the point of compression to the axilla, shoulder, arm, forearm, and hand. Paresthesias are usually aggravated at night. Motor abnormalities include weakness and muscle atrophy. Vascular involvement presents as pallor, pulselessness, and coolness of the affected side; venous obstruction is marked by edema and cyanosis. An elevated arm stress test (EAST) is used for screening purposes. Chest x-ray, MRI, and angiography help in diagnosing the cause of compression. Conduction velocity studies identify the site of compression. Overhead pulley exercises, shoulder bracing and other postural changes help to relieve compression.
Polymyelgia Rehumetica: characteristic findings are age of patient greater than 50, morning stiffness for 30 minutes or more and present for more than a months and it involves shoulder and hips. ESR is greater than 40 mm/hour. Most commonly pain and stiffness occur in a shoulder/trapezius, hip/girdle distribution. The diagnosis is supported by an elevated ESR or C-reactive protein (CRP).
The treatment of choice for polymyalgia rheumatica is with low-dose prednisolone. NSAIDs may be needed to treat mild discomfort when glucocorticoids are being tapered. Polymyalgia rheumatica may be present alone or in 40-50% cases, it is associated with giant cell arteritis. High-dose prednisolone therapy is used when giant cell arteritis is present. Temporal artery biopsy is required to confirm the diagnosis of giant cell arteritis. Features of giant cell arteritis include headache, jaw-claudication, transient visual loss and palpable tender arteries of scalp, neck or extremities.
Fibromyalgia: Presence of diffuse musculoskeletal pain, multiple tender points with no joint swelling/pain, usually makes the diagnosis. Initial evaluation should include a Complete blood count, ESR, Thyroid function tests (TFTs) and enzymes (CK). Hypothyroidism typically can present like this. It is far more common in females and usually presents between 35-55.
Hypothyroidism may be very difficult to differentiate from fibromyalgia, as it is often associated with generalized aches, fatigue, and disturbed sleep. So, TFTs should be routinely obtained during the initial evaluation of patients with suspected fibromyalgia.
Fibromyalgia is not an inflammatory condition and therefore NSAIDs such as naproxen or glucocorticoids have not been shown to be very effective in its treatment.
Tricyclic antidepressants like Amitriptyline have been shown to be effective in the treatment of fibromyalgia and they are usually given in low doses at nighttime. To relieve the pain, non-narcotic analgesics like acetaminophen can be given during the daytime. The only other drug other than tricyclic antidepressants studied extensively in fibromyalgia is cyclobenzaprine. So either amitriptyline or cyclobenzaprine are the initial drugs of choice. Narcotic analgesics like Oxycodone need to be avoided. Selective serotonin reuptake inhibitors (SSRIs) are added when disease is refractory to the above medicine. When patient feels better with the pharmacological treatment exercise program is initiated. The other treatment that can be tried in refractory cases are trigger point injections.
Chronic fatigue syndrome: although resembles Fibromyalgia, patients mostly complain of extreme fatigue and not body aches. No trigger points can be demonstrated; symptoms should be present for at least 6 months to make the diagnosis of chronic fatigue syndrome.
Gout: Gout results from deposition of monosodium urate crystals into the joint space. Nearly all patients with gout are hyperuricemic, but only 5% of patients with hyperuricemia have gout. Gout is an exquisitely painful monarthritis, but may be accompanied by polyarticular involvement. There may be fever. Most attacks subside after days or weeks. Gout usually occurs in the big toe, but in patients with untreated hyperuricemia, nodular gout may occur in the ear, ulnar surface of forearm, achilles tendon, and fingers. The sudden onset of pain and swelling in the metatarsophalangeal joint is suggestive of podagra, the common site of involvement in gout. This is more common in patients with myeloproliferative disorders, chronic renal disease, psoriasis, hemoglobinopathies and using medications such as thiazide, niacin etc. This is a metabolic disorder associated with hyperuricemia either due to over production or under secretion of uric acid. The demonstration of needle shaped urate crystals in the synovial fluid is diagnostic of gout; hence a joint fluid aspirate should be done. You should never leave a tender inflamed joint without aspiration. Tophi are nodular deposits of monosodium urate monohydrate crystals with associated foreign body reaction. Serum uric acid levels tend to be normal in some patients with acute gout, hence not very helpful in diagnosis.
In patients with frequent attacks of acute gouty arthritis not controlled by colchicine, a 24-hour uric acid levels in urine is determined. This evaluates whether hyperuricemia is due to over production or under secretion of uric acid. A value of less than 800 mg/day suggests under secretion. A value of more than 800 mg/day suggests over production. Hence accordingly a uricosuric agent or a xanthine oxidase inhibitor is added to the patient’s medication. The goal is to maintain serum uric acid levels to less than 6 mg/dL.
Pseudogout: is characterized by acute inflammatory changes of the large joints, most commonly the knee joint. It is associated with fever, chills, and leukocytosis. Synovial fluid analysis may show a leukocyte count as high as 100,000/cmm. Do not confuse with septic arthritis. Gram stain is usually negative in pseudogout. Episodes may be precipitated by surgery or trauma and can last up to 10 days if not treated. The diagnosis is confirmed by the finding of positively birefringent calcium pyrophosphate crystals and articular calcification.
Hemochromatosis is the only condition that has been found to be clearly associated (50%) with pseudogout. Pseudogout also occurs more frequently in patients with transfusion hemosiderosis. Other disorders associated with pseudogout include hyperparathyroidism, hypomagnesemia, hypophosphatemia and hypothyroidism.
Polymyositis: is an inflammatory muscle disease of unknown etiology. It presents as proximal weakness characterized by difficulty ascending and descending stairs, combing hair, kneeling down etc. There are no skin rash or scaly patches as seen in dermatomyositis. Though some patients present with muscle pain and tenderness, it is not very typical in majority. These patients have dysphagia due to involvement of the striated muscles of the upper pharynx. It shows endomysial infiltration of the inflammatory infiltrate. These conditions may occur alone or in association with a variety of neoplasms such as: breast, ovary, lung, prostate, or colon cancer. Suspected patients should have measurement of serum creatine kinase and aldolase levels. Muscle biopsy is the best diagnostic study for polymyositis.
Generally, polymyositis and dermatomyositis respond well to prednisone therapy. Cases associated with neoplasia respond less well, but may improve after removal of the associated tumor. In case the patient fails to improve on or cannot tolerate the side effects of corticosteroid therapy, immunosuppressive agents such as methotrexate may be effective.
Systemic sclerosis: is a chronic autoimmune disorder, which is characterized by fibrosis of skin and internal organs. There are two forms of systemic sclerosis: 1) limited, which is more common and 2) diffuse which occurs only in 15-20% of cases. The most characteristic findings of this disease are prominent vasomotor changes, fibrosis and subsequent atrophy of the skin, subcutaneous tissue, muscles, and internal organs, and immunologic changes. It is caused by excessive collagen deposition in the skin and internal organs. The affected organs and systems include the skin, heart, lungs, gastrointestinal tract, kidneys, muscle, joints, and nervous system. Raynaud’s phenomenon is a common finding, with fingers going through blanching, cyanosis, and erythema, accompanied by substantial pain. Subsequently, small, painful trophic ulcerations with or without subcutaneous calcification develop on the fingertips. Extensive disease may cause gangrene of the distal digits. Renal failure and pulmonary fibrosis are the most common causes of death. The characteristic antibody detected in patients with systemic sclerosis and interstitial lung fibrosis is antibodies against DNA topoisomerase I (Scl 70). Antibodies to U3-RNP are associated with systemic sclerosis and particularly correlate with progression to pulmonary hypertension.
CREST syndrome: is a form of scleroderma that is localized. In this form hardening of the skin is limited to the face and hands whereas in the diffuse form it extends to involve the trunk and proximal extremities. CREST is an acronym for the cardinal features of this syndrome Calcinosis, Raynaud’ s phenomena, Esophageal dysfunction, Sclerodactyly, and Telangiectasias. Calcinosis presents as nodular swellings, which may be symptomatic or asymptomatic. If symptomatic it presents with pain and tenderness, sometimes it may ulcerate and exude a white chalky fluid. X-ray of the involved area shows calcifications. CREST syndrome has a much better prognosis than diffuse scleroderma. These patients do not develop interstitial lung disease or renal failure as in diffuse form.
Eosinophilic fasciitis: also presents with diffuse skin changes similar to systemic sclerosis. The inflammation is limited to the fascia and does not involve the epidermis or the dermis. These patients do not have Raynaud’ s phenomena.
Eosinophilia myalgia syndrome: is seen in patients on tryptophan used for insomnia and pre menstrual syndrome. It is an essential amino acid and is currently banned by the food and drug administration. These patients develop diffuse myalgia and skin changes in the form of hives, generalized swelling and induration of the limbs.
Staphylococcal toxic shock syndrome: is caused by exotoxin and is characterized by fever, macular erythroderma of palms and soles, vomiting, diarrhea and hypotension. Use of highly adsorbent tampon is an important risk factor. Streptococcal toxic shock syndrome presents in a similar manner and follows invasive streptococcal infections.
Mixed Connective Tissue Disease (MCTD): Diagnosis of Mixed Connective Tissue Disease (MCTD) is made when serology shows positive anti-RNP antibodies and clinical features of SLE, polymyositis and systemic sclerosis are present simultaneously. These features include swollen hands, synovitis, myositis, acrosclerosis, and Raynaud’s phenomenon. If three of these features are present, along with positive anti-RNP, diagnosis of MCTD is established. This occurs in a much younger population. It later evolves into any of the rheumatic disorders.
Dermatomyositis: an inflammatory myositis characterized by edema, dermatitis, and muscular inflammation with degeneration. With or without skin lesions, weakness of proximal muscle groups is characteristic. The cutaneous signs of dermatomyositis may precede or follow the development of myositis by weeks- years. Periorbital edema and a purple-red discoloration of the upper eyelids, sometimes associated with scaling (heliotrope erythema) are the most common skin manifestations. Erythema and scaling may be particularly prominent over the elbows, knees, and the interphalangeal joints. A reddish-purple, scaling eruption occurs over the metacarpal-phalangeal joints, elbows, and knees (Gottron's sign) with flat-topped, violaceous papules pathognomonic of dermatomyositis over the metacarpal-phalangeal joints (Gottron's papules). Hyperkeratosis, scaling, fissuring, and hyperpigmentation over the fingertips, sides of the fingers, and palms is referred to as mechanic’s hands, and has been associated with antisynthetase antibodies in 70% of patients. Photosensitivity is common, and most skin lesions are present in sun-exposed areas. Pruritus is variable, from none to devastating. Intermittent fever, malaise, anorexia, arthralgia, and marked weight loss are present at this stage. In severe cases, early and extensive muscular weakness occurs with acute swelling and pain. It most frequently involves the shoulder girdle and pelvic region in a symmetric way. Patients may not be able to raise their arms to undress or comb their hair, or they may have difficulty rising from a sitting position, squatting, or climbing stairs. Other involved muscle groups may present with difficulty swallowing, talking, or breathing. In the terminal phase of the disease, cardiac involvement with failure may be present. The following criteria are used to define dermatomyositis/polymyositis:
Suspected patients should have measurement of serum creatine kinase and aldolase levels. Specific diagnosis is usually made by muscle biopsy. These patients should have a complete metastatic workup to rule out malignancy. It includes PAP smear, mammogram, CT chest / abdomen / pelvis, and colonoscopy.
Prednisone is the mainstay of treatment, along with steroid-sparing immunosuppressive agents such as methotrexate, azathioprine, and cyclosporine.
SLE: a severe, life-threatening autoimmune connective tissue disease. Skin involvement occurs in 80% of cases and is likely to be very helpful in making the correct diagnosis. The features includes:
1.Systemic symptoms: Fatigue, fever, and weight loss.
2.Cutaneomucous symptoms: Malar rash, discoid rash, alopecia, oral or nasopharyngeal ulcers, and photosensitivity. The lupus band test is direct immunofluorescence of non–sun-exposed skin. A positive lupus band test correlates with aggressive course of systemic disease in patients with systemic lupus erythematosus. 3.Serositis: pericarditis, pleuritis
4.Nephritis: which can be
-Mesangial, which is characterized by focal and segmental glomerular involvement with an increase in mesangial cells;
-Focal proliferative form;
-Diffuse proliferative form, which is characterized by a "wire loop" pattern resulting from immune complex deposition and subsequent thickening of the glomerular basement membranes. It can result in irreversible changes and chronic renal failure
-Membranous form, which is similar to primary membranous glomerulonephritis.
5.Neurologic symptoms: Depression, psychosis, seizures, and neuropathy.
6.Non-erosive arthritis: Arthritis and arthralgias of SLE are migratory and asymmetrical.
7.Hematologic disease: Hemolytic anemia; leukopenia; lymphopenia; thrombocytopenia.
The serologic pattern of the disease is characterized by the presence of positive antinuclear antibodies; anti-dsDNA antibodies; anti-Sm antibodies;
SLE in some cases presents with renal involvement. Although SLE can cause several types of immune-mediated tissue damage and at the same time causes several well defined kidney diseases, it is believed that the main mechanism of kidney damage in SLE patients is immune complex-mediated. Immune complexes circulate in the blood and are deposited in renal glomeruli causing their damage via several mechanisms. One of these mechanisms includes complement activation. That’s why serum C3 level is decreased in immune complex-mediated kidney diseases such as SLE and poststreptococcal glomerulonephritis. When kidney is involved, a SLE renal biopsy is needed, especially in those cases where renal involvement is less severe with mild proteinuria and hematuria or when presentation is of nephrotic syndrome with bland urinary sediment. Therapy is then directed by pattern of glomerular involvement. Renal involvement is very common in patients of systemic lupus erythematosus (SLE). Renal biopsy shows involvement of kidneys in more than 90% patients of SLE. Abnormal urinalysis is present in more than 50 percent patients of SLE. Most common type of renal injury in patients of SLE is immune mediated glomerular injury that can be divided into six sub-groups. Type I is normal. Remaining five are as follows: Mesangial, focal proliferative, diffuse proliferative, membranous and sclerosing.
Mesangial is the earliest and the least severe form and is present in 10-20 percent of cases. Anti-DsDNA titers are not very high.
Focal proliferative is a severe form than Mesangial form and is present in 10-20 percent of cases. Urinalysis shows proteinuria and hematuria and serum creatinine is elevated. Histopathology shows proliferative changes with some areas of necrosis. Less than 50 percent of glomeruli are affected.
Diffuse proliferative is the most frequent pattern of renal involvement and unfortunately is the severest form. Hematuria, proteinuria, renal insufficiency, hypertension, marked hypocomplementemia and marked elevation of anti-DsDNA are all present. Histopathology shows changes that are similar but more severe than in focal proliferative type. More than 50 percent of glomeruli are affected. It has the worst of all prognosis.
Membranous glomerulonephritis is present in 10-20% cases of SLE and usually presents with nephrotic syndrome. Renal function is preserved. Basement membrane is thickened and subepithelial deposits are present. It has better prognosis than diffuse proliferative type.
Sclerosing type represents healing of previous inflammatory damage and presents with renal insufficiency and normal urinary sediment. Immunosuppressive therapy is not effective in such patients as there is no active inflammation.
Oral contraceptive pills use in SLE is considered safe if the patient is in remission. In patients with active nephritis, oral contraceptive pills shouldn’t be used as it can aggravate renal disease. Barriers method is used instead.
Anti phospholipid antibody syndrome: is characterized by recurrent arterial or venous thrombosis or recurrent fetal losses in the presence of anti phospholipid antibodies. Anti phospholipid antibodies are of three types. The first is responsible for false-positive syphilis serology. The second is lupus anticoagulant, which falsely elevates APTT. The third is anticardiolipin antibody. Antiphospholipid antibody syndrome is either primary or may be associated with other autoimmune disorders like SLE.
Sarcoidosis: Sarcoidosis is a systemic disease characterized by granulomatous inflammation of the lung. It is more common in African-American women in their third and fourth decades. It presents with cough, dyspnea, non-specific chest pain and skin lesions characteristic of erythema nodosum. It should be suspected in patients with bilateral ankle arthritis and bilateral hilar adenopathy on chest-x ray.
Sarcoidosis is basically two types: Acute and chronic. Lofgren’s syndrome is an acute form of sarcoidosis predominantly seen in Scandinavian, Irish, and Puerto Rican woman. The characteristic presentation consists of triad of arthritis (of ankles, knees, wrists or elbows), erythema nodosum and bilateral hilar adenopathy. Lung involvement is very rare in this acute form. Laboratory studies suggest inflammatory disease with elevated ESR, false positive rheumatoid factor, and antinuclear antibodies.
Heerfordt-Waldenstrom syndrome is another rare from of acute sarcoidosis consists of fever, parotid enlargement, anterior uveitis, and facial nerve palsy.
Chronic sarcoidosis has an insidious onset that develops over months. It mainly presents with respiratory complaints without constitutional symptoms. Very few patients have organs other than lung involved. It leads to permanent lung damage.
Systemic steroids are the drugs of choice for sarcoidosis and indicated in patients with disabling symptoms, organ dysfunction, organ derangements, and abnormal serum tests. Steroids effectively suppress the activated T helper-inducer cell processes occurring at the disease site. Asymptomatic sarcoidosis on the other hand does not require treatment.
Cyclosporine is mainly used in patients with extra thoracic sarcoidosis, not responding to systemic steroids.
Osteogenesis imperfecta—an autosomal dominant disease. The most common molecular defect is a mutation in one of the two genes encoding type I procollagen.
Type II procollagen gene mutation is found in patients with chondrodysplasia—an inherited skeletal disorders that cause dwarfism, ocular changes and cleft palate.
Type III procollagen gene mutation is found in patients with Ehlers-Danlos syndrome, that is characterized by hyperelasticity of the skin and hypermobile joints.
Paget’s disease: of the bone is characterized by excessive bone destruction and repair. It generally is asymptomatic and may be suspected by findings of elevated levels of alkaline phosphatase. The serum calcium is usually normal. Over a period of time 1-3% of individuals will develop osteosarcoma or sarcomatous changes. This will manifest itself as marked bone pain, new lytic changes and a sudden increase in alkaline phosphatase.
Wegener’s granulomatosis: is a granulomatous vasculitis-affecting upper and lower respiratory tracts as well as causing glomerulonephritis. Patient usually presents with hematuria, hemoptysis and features of upper respiratory tract or sinus involvement. Labs show elevated c-ANCA.
Churg Strauss syndrome: is a multisystem vasculitis disorder of unknown etiology that affects skin, kidney, nervous system, lungs, gastrointestinal tract and heart. There is a history of asthma followed by the development of fever and marked eosinophilia at which time asthma may become better. The disease is severe and requires treatment with glucocorticoids and sometimes with immunosuppressants.
Polyarteritis nodosa: involves medium sized arteries and most frequently organs include kidney, liver, gastrointestinal tract, skin, heart and peripheral nerves. It has been found to be associated with hepatitis B and C. Labs shows elevated P-ANCA.
Buerger’s Disease: It is an episodic, and segmental inflammatory process of small and medium sized arteries (thromboangiitis obliterans). It is characterized by occlusive disease of the arteries, migratory superficial thrombophlebitis, and Raynaud’s phenomenon. Usually one or two of these three conditions are present. Other important features are young age, males and history of smoking. Cessation of smoking is an important component of management.
Takayasu’s arteritis (Pulseless disease): is of unknown cause, and usually involves branches of the aortic arch. The symptoms caused by this disease are caused by cerebrovascular insufficiency, such as absent pulses in upper limb and neck bruit.
Giant cell arteritis: should always be suspected in a patient older than 50 years who presents with headache, visual problems and polymyalgia rheumatica. Such patients may also have weight loss, anemia and high ESR. Lab studies may shows elevated platelet counts and normocytic normochromic anemia. Therapy with high dose glucocorticoids is the treatment of choice and should be started immediately when diagnosis of giant cell arteritis associated with visual disturbances is strongly suspected. Blood samples can be drawn for ESR before the start of glucocorticoid therapy and temporal artery biopsy that is required to confirm the diagnosis needs to be done after starting steroid treatment. One should not wait for temporal artery biopsy before starting glucocorticoid therapy as any delay in the treatment may result in visual loss. Treatment with steroids does not affect the results of temporal artery biopsy. High dose steroid therapy is continued till the symptoms resolve and labs become normal.
Methotrexate is also effective in the treatment of giant cell arteritis but it is used only as a steroid-sparing agent and is not indicated at this time.
Thoracic aortic aneurysm, that is 16 times more common in patients of giant cell arteritis than the general population, is a serious complication and such patient need to have continuous monitoring. This aneurysm may dissect and prove to be fatal. The possible explanation for the development of aneurysm is disruption of collagen and elastin by chronic aortitis.
Cryoglobulinemia:Hepatitis-C virus infection account for at least 80% of the vasculitis cases associated with mixed cryoglobulinemia. Therefore all such patients should be tested for hepatitis C virus (HCV) infection by serum HCV antibody or HCV RNA. If these tests are negative, cryoprecipitate can be tested for HCV antibody or HCV RNA.
Only few cases (case reports) of mixed Cryoglobulinemia are due to infection with HBV virus and HIV virus, therefore testing for these viruses is not routinely indicated in patients of mixed cryoglobulinemia.
Cyclophosphamide toxicity: Cyclophosphamide produces many adverse effects, the most frequent of which include alopecia, sterility, amenorrhea, nausea, vomiting diarrhea, mucositis, stomatitis, acute hemorrhagic cystitis, bladder carcinoma, and blood dyscrasias. The proposed mechanism for acute hemorrhagic cystitis and bladder cancer is irritation by acrolein that is a metabolite of cyclophosphamide. Taking plenty of fluids, voiding frequently and avoiding the drug at night can prevent this side effect. Use of mesna is sometimes indicated for this purpose.
Hydroxychloroquine is the safest drug for systemic lupus erythematosus (SLE). However, rarely, it may cause serious eye disease including macular degeneration; so eye examinations at 6 months to 1 year intervals should be performed in all such patients who are taking this drug. Visual field defects, impaired color vision, bull’s eye pattern on funds and corneal whirls can all occur with its use.
The most common side effects of hydroxychloroquine are allergic skin reactions and nausea but they are not serious ones. The incidence of nausea can be reduced if it is taken after meals. Contraindications to its use include G6PD deficiency, porphyria cutanea tarda, liver failure and hepatic failure. It is also need to be avoided in pregnancy.
Though it may cause hepatic failure, deafness and blood dyscrasias they are very rare and can be monitored by liver function tests (LFTs), hearing examination and CBC with differential respectively. This monitoring is not recommended regularly.
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