Keywords
ANCA-associated vasculitis
Anti-glomerular basement membrane
Plasmapheresis
How to Cite
Abstract
Background: Pulmonary-renal syndrome is a rare but severe condition characterized by simultaneous rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. It is subclassified into four types: 1) Goodpasture Syndrome, 2) Immune complex mediated, 3) Associated with ANCA vasculitis (Pauci-immune), and 4) Dual positivity of ANCA and anti-GBM antibodies. Type 4 is the least common of the subtypes and is gaining awareness for its distinct prognosis and management.
Purpose: To present a rare case of pulmonary renal syndrome with double positivity for anti-neutrophil cytoplasmic antibodies (ANCA) and anti-glomerular basement membrane (anti-GBM) antibodies, highlighting the clinical presentation, radiological findings, and proposed management.
Case presentation: We present a 23 years old male with flu-like symptoms that progressed to hemoptysis, dyspnea, and peripheral edema. His renal biochemical tests and imaging were consistent with rapidly progressive glomerulonephritis, and lung images showed diffuse alveolar hemorrhage, confirming pulmonary-renal syndrome. Treatment included plasma exchange, hemodialysis due to refractory fluid overload, and steroids. Laboratory tests confirmed the presence of anti-membrane basal antibodies and anti-MPO antibodies. Renal biopsy revealed cellular crescents and a necrotizing pattern without chronic features.
Discussion and conclusion: Dual positivity for anti-MBG and anti-MPO antibodies is a rare cause of pulmonary-renal syndrome. Despite its low incidence and the overlapping features of the two conditions, it has distinct prognosis and recurrence patterns. Aggressive immunosuppressive treatment has shown promising results. Early detection and timely treatment are crucial for improving clinical outcomes.
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