9-28: Alveolar Hemorrhage Syndromes

Diffuse alveolar hemorrhage may occur in a variety of immune and nonimmune disorders. Alveolar infiltrates on chest radiograph, dyspnea, anemia, hemoptysis and, occasionally, fever are characteristic. Rapid clearing of diffuse lung infiltrates within 2 days is a clue to the diagnosis of diffuse alveolar hemorrhage (eFigure 9–23). Pulmonary hemorrhage can be associated with an increased DLCO, although this test is infrequently obtained. Sequential BAL on bronchoscopy is the preferred method for diagnosis with lavage aliquots becoming progressively more hemorrhagic.

Causes of diffuse immune alveolar hemorrhage include anti-basement membrane antibody disease (Goodpasture syndrome), granulomatosis with polyangiitis, systemic necrotizing vasculitis, pulmonary capillaritis associated with idiopathic rapidly progressive glomerulonephritis, SLE, and other vasculitic and collagen vascular diseases. Nonimmune causes of diffuse hemorrhage include coagulopathy, mitral stenosis, necrotizing pulmonary infection, drugs (penicillamine), toxins (trimellitic anhydride), and idiopathic pulmonary hemosiderosis.

Idiopathic pulmonary hemosiderosis is a disease of children or young adults characterized by recurrent pulmonary hemorrhage; iron deficiency is typical. It is frequently associated with celiac disease. Treatment of acute episodes of hemorrhage with corticosteroids may be useful. Recurrent episodes of pulmonary hemorrhage may result in interstitial fibrosis and pulmonary failure.