Abstract
Pulmonary-renal syndrome describes a dramatic presentation of multi-system autoimmune disease characterized by diffuse pulmonary hemorrhage and glomerulonephritis. Autoantibodies to neutrophil cytoplasmic antigens (ANCA) associated systemic vasculitis and anti-glomerular basement membrane (GBM) disease account for over 60 and 20%, respectively, and the presence of circulating ANCA or anti-GBM antibodies or both, provides a tool for diagnosis, classification and monitoring of disease. The close relation between ANCA and pauci-immune alveolar and glomerular capillaritis indicate a pathogenic role for these autoantibodies, further supported by evidence from experimental models. Respiratory failure due to pulmonary hemorrhage is the most frequent vasculitic cause of referral to an intensive care unit, where the course is often complicated and prolonged; it carries a mortality of 50% due to refractory hypoxia or secondary infection, and many survivors progress to end-stage renal failure. Aggressive treatment with corticosteroids, cyclophosphamide and plasma exchange can induce sustained remission, but success is dependent on rapid diagnosis, facilitated by the use of serological markers, careful monitoring of therapy, and avoidance of infection.
Published Version
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