Pulmonary manifestations of the clinical syndrome of acute glomerulonephritis and lung hemorrhage.

The pulmonary manifestations at the time of initial diagnosis were reviewed in 45 patients with the clinical syndrome of acute glomerulonephritis and lung hemorrhage. Initial pulmonary radiographic appearances encompassed a wide variety of abnormalities, and alveolar hemorrhage could not be reliably differentiated from other causes of pulmonary infiltration. The diseases underlying the syndrome included antiglomerular basement membrane (anti-GBM) disease (8/45), a systemic vasculitis (25/45), and idiopathic glomerulonephritis with idiopathic lung hemorrhage (12/45). A variety of acute pulmonary complications were seen, the most common being acute respiratory failure (13/45). Mortality directly due to pulmonary disease was uncommon (3/45 died from fulminant lung hemorrhage). Most deaths were from extrapulmonary manifestations of the underlying disease or infection. Follow-up studies in 22 patients 6 months after initial presentation indicated that although respiratory symptoms (3/22) or pulmonary radiologic abnormalities (5/22) were uncommon, the majority of patients had residual abnormalities on pulmonary function testing (16/22). Thus, the syndrome of alveolar hemorrhage with nephritis is associated with several distinct categories of underlying disease process. The initial chest radiograph is of limited value in differential diagnosis. Although a variety of acute and chronic respiratory complications may be anticipated and contribute significantly to morbidity, mortality is predominantly due to extrapulmonary progression of disease or infection complicating immunosuppressive therapy.