doc_clotaire
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A 31-year-old previously healthy man comes to your office because of a 2-week history of low-grade fevers, weight loss, malaise, nocturnal tightness in his chest, and shortness of breath. He also reports a small amount of leg swelling and scrotal swelling over this time. Prior to 2 weeks ago, he denies any recent illnesses, sick contacts, or travel. He also denies any hemoptysis or sinus infections. His temperature is 38.0 C (100.2 F), blood pressure is 170/95 mm Hg, pulse is 77/min, and respirations are 14/min. Physical examination shows trace bilateral lower extremity and scrotal edema. Bilateral wheezes are also appreciated. A chest x-ray is unremarkable. Laboratory studies show:
Sodium 141 mEq/dL Potassium 5.1 mEq/dL Chloride 98 mEq/dL Bicarbonate 21 mEq/dL Urea nitrogen, serum 21 mg/dL Creatinine 2.5 mg/dL Glucose 97 mg/dL Serum eosinophils mildly elevated Urinalysis Color clear Specific gravity 1.020 Osmolality 55 mOsmol/kg Leukocyte esterase negative Nitrite negative Protein 2+ Blood 4+ Microscopic many red cell casts. 24 hour urine protein collection 1900 mg The laboratory finding that would support the most likely diagnosis is
A. anti-glomerular basement membrane antibody
B. antistreptolysin titer (ASO)
C. cANCA
D. elevated serum IgA levels
E. pANCA
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| new_n_lost
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D. elevated serum IgA levels>> IgA Nephropathy ??
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| farnsworth
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E - The patient is suffering from Churg-Strauss syndrome
(which is associated w/ eosinophilia, allergic asthma, or induced by asthma medication, e. g. Montelukast)
why not:
A) Goodpasture syndrome (hemoptysis!)
B) post-streptococcal glomerulonephritis (infection!)
C) Wegener's granulomatosis (sinusitis!)
D) IgA nephropathy (the patient has eosinophilia, otherwise this would fit, too)
Honestly, I had to look this up (in the Great Book of Big, Bad Nephrological Diseases 'n Stuff)
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| guangyu
Forum Elite
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E
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| doc_clotaire
Forum Guru
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Explanation:
The correct answer is E. This patient likely has Chrug-Struass syndrome. Chrug-Struass syndrome is a nephritic syndrome, which is associated with eosinophilia and asthma. This patient has nephritic syndrome as evidenced by edema, hypertension, and hematuria. Asthma is suggested by his nocturnal chest tightness and shortness of breath. Laboratory results reveal elevated eosinophils, which is typical in this condition. Chrug-Strauss is associated with a positive pANCA.
[font size="3"]Goodpastures syndrome is typically defined as a combination of nephritic syndrome and pulmonary hemorrhage, although some patients have nephritic syndrome alone. The disease is mediated by antibodies to the glomerular basement membrane (choice A) and therefore those are the laboratory results often associated with the disease.
ASO (choice B) is associated with post infectious glomerulonephritis. Patients present with oliguria, hypertension, and edema. They classically complain of Coca-Cola colored urine. These infections classically occur 1-3 weeks after infection with nephritogenic group A streptococci (step throat or cellulitis).
Wegner granulomatosis is similar to Chrug-Strauss but respiratory involvement such as nasal septal perforation or sinus problems are typical presenting signs. Wegners doesn't have an association with asthma or increased serum eosinophils. The test associated with Wegners is cANCA (choice C).
Bergers syndrome (IgA nephropathy) presents in a similar manner to post-infectious glomerulonephritis but there is no latent period between infection and kidney involvement. These patients typically have gross hematuria after a viral illness. About 50% of these patients will have an elevated IgA (choice D).[/font]
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