A 30-year-old male presents with recurrent episodes of hemoptysis and glomerulonephritis with progressive renal failure. Urinalysis demonstrates dysmorphic RBCs and RBC casts. Serum serology reveals high titers of anti-glomerular basement membrane (anti-GBM) antibodies. What is the diagnosis?
Answer and explanation
Correct answer: C. Goodpasture Syndrome (Anti-GBM Disease)
- Step 1: Goodpasture syndrome (Anti-GBM disease) is an autoimmune disorder caused by autoantibodies directed against the alpha-3 chain of type IV collagen.
- Step 2: This cross-reacts with basement membranes in both the pulmonary alveoli (causing alveolar hemorrhage/hemoptysis) and the renal glomeruli (causing rapidly progressive crescentic glomerulonephritis).
Answer
Goodpasture Syndrome (Anti-GBM Disease).
Why not the others: 'Wegener Granulomatosis (GPA)' is associated with c-ANCA (anti-PR3) antibodies, necrotizing granulomas of the upper/lower respiratory tract, and lacks anti-GBM antibodies; 'Churg-Strauss (EGPA)' features p-ANCA, severe asthma, and peripheral eosinophilia; 'Alport Syndrome' is an X-linked genetic disorder involving type IV collagen mutations causing sensorineural deafness and hematuria.
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