[Pulmonary-renal syndrome]

Pulmonary-renal syndrome is a potentially life-threatening disorder, characterized by diffuse alveolar hemorrhage on the basis of pulmonary capillaritis in conjunction with rapidly progressive glomerulonephritis. Pulmonary-renal syndrome can originate from various systemic autoimmune diseases. ANCA-associated vasculitides account for approximately 60%, Goodpasture's Syndrome for approximately 20% of the cases. Fulminant pulmonary capillaritis can result in acute respiratory failure, more subtle courses are only detected by bronchoalveolar lavage. Renal biopsy displays extracapillary proliferating glomerulonephritis and renal immunohistology facilitates detection of the underlying systemic disease. By accelerating the diagnosis of the specific underlying disease, autoantibody testing fosters rapid initiation of treatment and thereby significantly improved the prognosis of pulmonary-renal syndrome. Intense immunosuppression with cyclophosphamide and glucocorticoids, augmented by plasmapheresis in the event of Goodpastures' syndrome, is the mainstay of therapy. Supportive measures as temporary ventilation and hemodialysis have further reduced mortality.