Alveolar Hemorrhage Syndromes

KEY FEATURES

• Diffuse alveolar hemorrhage may occur in a variety of immune and nonimmune disorders

• 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

  • Systemic lupus erythematosus

  • Other vasculitic and collagen vascular diseases (see Part 22).

• Nonimmune causes include

  • Coagulopathy

  • Mitral stenosis

  • Necrotizing pulmonary infection

  • Medications (penicillamine)

  • Toxins (trimellitic anhydride)

  • Idiopathic pulmonary hemosiderosis (usually seen in children or young adults)

• Idiopathic pulmonary hemosiderosis

  • Characterized by recurrent pulmonary hemorrhage

  • Frequently associated with celiac disease

• Recurrent episodes of pulmonary hemorrhage may result in interstitial fibrosis and respiratory failure

CLINICAL FINDINGS

• Acute dyspnea, anemia, hemoptysis and, occasionally, fever are characteristic]

• Iron deficiency is typical with idiopathic hemosiderosis

DIAGNOSIS

• Chest radiograph or CT chest with rapid clearing of diffuse lung infiltrates

• Sequential bronchoalveolar lavage on bronchoscopy

• DLCO

  • Not frequently obtained

  • May be increased

TREATMENT

• Corticosteroids may be useful for acute episodes of hemorrhage