idiopathic pulmonary hemosiderosis (hemorrhage)

Etiology

• unknown, a diagnosis of exclusion
• implicated factors
  • heritable defect
  • immunologic disorder associated with antibodies to cow's milk
  • cold agglutinins
  • increased serum IgA
  • viral infection
  • primary disorder of airway epithelial cells
  • structural defect in pulmonary capillaries
• disease associations
  • idiopathic thrombocytopenic purpura (ITP)
  • autoimmune hemolytic anemia
  • non-tropical sprue (celiac disease)
• pediatric form of the disease thought to be related to spores growing in humid basements

Epidemiology

• most cases begin in childhood
• in childhood, the male:female ratio is 1:1
• in adults, the male:female ratio is 3:1

Pathology

• recurrent intra-alveolar hemorrhage
• hemosiderin-laden macrophages
• fibrosis & cor pulmonale may occur
• kidneys are not involved

Clinical manifestations

• chronic cough
• intermittent hemoptysis
• fever
• weight loss
• generalized lymphadenopathy (25%)
• hepatosplenomegaly (20%)
• clubbing (15%)
• course of the disease is generally prolonged
• often fatal

Laboratory

• evidence of iron-deficiency anemia
  • complete blood coutn (CBC)
  • iron studies: serum iron, TIBC, serum ferritin
  • low reticulocyte count for degree of anemia
• cold agglutinins in some patients
• eosinophilia (10%)

Radiology

• chest X-ray
  • transient blotchy, perihilar infiltrates in mid & lower lung fields
  • small nodules may be observed
• computed tomography:
  • intrathoracic lymphadenopathy in 25% of patients

Management

• blood transfusions
• iron therapy
• corticosteroids
• cytotoxic agents may be useful
• prognosis: 5 year mortality is 30%

More general terms

• pulmonary siderosis
• hemosiderosis

Additional terms

• alveolar hemorrhage