Idiopathic Pulmonary Hemosiderosis
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Contents |
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
- 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
-
- transient blotchy, perihilar infiltrates in mid & lower lung fields
- small nodules may be observed
- 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
Additional Terms
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 786
