frank100
Forum Guru
Topics: 48
Posts: 586
|
A 63 y/ white female is admited to the hospital complaining of hemoptysis and shortness of breath. She had been well until 3 months ago, when she noted vague symptoms of fatigue and a 10 pound unintentional weight loss. Past medicall hystory is notable only for osteoporosis. Her current symptoms began on the day of presentation with the espectoration of >200 mL of red blood in the emergency department. On physical examination the patien is in marked respiratory distress with a respiratory rate of 44. O2 sat: 78% on room air and 88% on nonbreather mask. pulse is 120 beats per minute. A blood pressure of 70/110. there are diffuse crackles thoughout both lung fields, and the cardiac examination is significant only for a regular tachycardia. there are no rashes or joint swellings. Laboratory studies reveal a hemoglobin of 10.2 mg/dl with a MCV: 88. the WBC: 9760/mm3. BUN: 78, AND CREATININE 3.2. urianalysis shows 1+ proteinuria, moderate hemoglobin, 25 to 35 RBC and ocasional RBC casts. CT shows diffuse alveolar infiltrates consistent with alveolar hemorrhage. the antimyeloperoxidase titer is positive at 126 U/ml (normal <1.4 U/mL). what is the most likely diagnosis?
a) goodpasture´s disease
b) wegener´s granulomatosis
c) Microscopic polyangiitis
d) polyarteritis nodosa
e) cryoglobulinemia
|
| GDS2008
Forum Elite
Topics: 9
Posts: 144
|
most likely b) wegener´s granulomatosis
|
| mazinger
Forum Guru
Topics: 46
Posts: 918
|
agree with your answer
You almost got me here frank... At the begining it seemed like a GPastures but that diagnosis doesnt fit to a 63 yo woman, and the antibodies part anti-myeloperoxidase is another name for ANCA...
Good question, and you made me think a lot...
___________________
original mazinger z
|
| frank100
Forum Guru
Topics: 48
Posts: 586
|
the answer is not B.
|
| MAZI
Forum Elite
Topics: 8
Posts: 245
|
AND THEN A
YES
|
| humbty
Forum Elite
Topics: 24
Posts: 135
|
I would say C microscopic polyangitis.
Its similar to wegeners but has no granulomatous lesions, also ANCA positive. pulmonary capillarities and glomerulonephritis are typical.
|
| yasmeen
Forum Guru
Topics: 70
Posts: 942
|
E CRYOGLOBINEMIA
|
| frank100
Forum Guru
Topics: 48
Posts: 586
|
the answer is C:
microscopic polyangiitis is a small vessel vasculitis associatedwith ANCAs OF THE PERINUCLEAR TYPE. MPA was recognized as a discrete entity in 1992, when it was distinguished from polyarteritis nodosa because of the involvement primarily of the small vessels. 12% of cases present primarily with difuse alveolar hemorrhage. MPA is distinct fron wegener´s granulomatosis because it does not induce granulomatous inflamation. the glomerulonephritis associated with MPA IS PAUCI-IMMUNE, showing a lack of immunoglobulin deposition. p-ANCA staining is positive in 75% of patients with MPA, with antimyeloperoxidase antibodies bing the traget if the immunofluorescence staining pattern of the p-ANCA. therapy begins with high dose steroids and often requires the addition of cytotoxic therapy and cyclophosphamide. survival rate 5 years is 74%, however the disease tends to be chronic, with at least 34% relapse rate
|
| msyamp
Forum Fanatic
Topics: 60
Posts: 1,462
|
frank pleeez tell the answer and explantation. I cant wait. this is really tough
___________________
If you think you can You can! If you think you cant you are right again!!
|
| mazinger
Forum Guru
Topics: 46
Posts: 918
|
Like they say in my country.. "Vishiii!" 
Cool question, cool answer!
___________________
original mazinger z
|
| GDS2008
Forum Elite
Topics: 9
Posts: 144
|
I agree that this can be either Wegner's or microscopic polyangiitis. The latter involves not only the renal and pulmonary system, but can also other organ systems like Musculoskeletal, skin, GI, occular and even CNS. On the other hand, we know that Wegner's only invovles Renal and pulmonary systems. Of course, the deifinitive diagnosis can onlt be established on the basis of a biopsy (whihc is not given here). Therefore, if a similar question appears on the exam with just pulmonary and renal findings (without mention of other organ/systems) and no biopsy findings, what would one pick if asked for "the most likely diagnosis based on signs and symptoms"? I will be inclined to pick Wegner's Granulomatosis. Any suggestions?
IS there something specific in the question that points towards microscopic polyangiitis and not Wegner's??
|
| humbty
Forum Elite
Topics: 24
Posts: 135
|
I would say the CT is much more typical for microscopic polyangitis than for wegeners.
|
| GDS2008
Forum Elite
Topics: 9
Posts: 144
|
yes U r right -- also, i was wondering if RBC casts are seen in Wegner's??
|
| anjushree
Forum Guru
Topics: 64
Posts: 386
|
WHAT IS THE T/t
when u r giving cyclophosphamide ,prophylaxis against which org is essential
|
| AAzad
Forum Guru
Topics: 57
Posts: 460
|
hard one
___________________
AAzad
|
| liwei
Forum Junior
Topics: 7
Posts: 72
|
anti-myeloperoxidase = P-ANCA
so, the answer is C which has similar symptoms of Wegener
|
| fox
Forum Guru
Topics: 70
Posts: 727
|
anjushree wrote:
WHAT IS THE T/t
when u r giving cyclophosphamide ,prophylaxis against which org is essential
could u tell us watz the answer anju?
___________________
Aim High
|
| anjushree
Forum Guru
Topics: 64
Posts: 386
|
CMDT----CYCLOPHOSPHAMIDE is the t/t-----following induction of remission,azathioprine replaces cyclophosphomide
whenever cyclophos is used ----prophylaxis of PCP with TMX-SMZ OR DAPSONE IS USED
|
| anjushree
Forum Guru
Topics: 64
Posts: 386
|
MCC of pul-renal syn?
|
| fox
Forum Guru
Topics: 70
Posts: 727
|
Goodpastures?
___________________
Aim High
|
| AAzad
Forum Guru
Topics: 57
Posts: 460
|
Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies observed in patient serum in a wide range of vasculitic diseases including Wegener's granulomatosis, Churg-Strauss syndrome, idiopathic crescentic glomerulonephritis, and microscopic polyangiitis where a renal-pulmonary syndrome including pauci-immune necrotizing crescentic glomerulonephritis (NCGN) and pulmonary capillaritis can be observed .
Proteinase 3 and myeloperoxidase (MPO), both myeloid lysosomal enzymes, have been identified as the primary target antigens for ANCA in those disorders . It has been well established that detection of ANCA is useful in the diagnosis and monitoring of systemic vasculitic disorders . However, the pathophysiological role of ANCA in their associated diseases has not been fully elucidated. In vitro experiments have shown that ANCA can activate neutrophils leading to oxygen radical production and degranulation of proteolytic lysosomal enzymes . In addition, ANCA promote the adhesion of neutrophils to monolayers of endothelial cells and may cause vascular injury by activating primed neutrophils to induce endothelial cell lysis . Although in vitro observations suggest a pathogenic role for ANCA, their in vivo relevance is not clear. Studying the pathophysiological role of ANCA in vivo reported on an animal model for anti-MPO-associated pauci-immune NCGN. pauci-immune NCGN is induced in MPO-immunized rats by renal perfusion of MPO, proteolytic enzymes, and H2O2 .
In most cases of ANCA-associated vasculitides, disease manifestations are not confined to the kidneys . Especially pulmonary involvement is frequently reported . In ANCA-associated vasculitis a similar pathogenesis may underlie the disease manifestations in both lungs and kidneys.
___________________
AAzad
|
| anjushree
Forum Guru
Topics: 64
Posts: 386
|
CMDT----MCC of renal pul syndrome is microscopic polyangiitis
|
| prathapdoctor
Forum Elite
Topics: 12
Posts: 406
|
is it, thank you for putting forward a very good point
|
|
| |
| | | | | | | | | | | | | | | | | | | | | | | |
