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Idiopathic Pulmonary Hemosiderosis

By Marvin I. Schwarz, MD, James C. Campbell Professor of Pulmonary Medicine, University of Colorado Denver

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Patient Education

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease that causes recurrent diffuse alveolar hemorrhage with no detectable underlying disorder; it occurs mainly in children < 10 yr. It is thought to be due to a defect in the alveolar capillary endothelium, possibly due to autoimmune injury. Many affected patients have celiac disease.

Symptoms and Signs

Symptoms and signs of idiopathic pulmonary hemosiderosis in children include recurrent episodes of dyspnea and cough, particularly nonproductive cough initially. Hemoptysis occurs later. Children with IPH may present with only failure to thrive and iron deficiency anemia. The most common symptoms in adults are exertional dyspnea and fatigue due to pulmonary hemorrhage and iron deficiency anemia.

Diagnosis

  • Bronchoalveolar lavage

Diagnosis of idiopathic pulmonary hemosiderosis involves demonstration of a combination of characteristic clinical findings, iron deficiency anemia, and hemosiderin-laden macrophages in bronchoalveolar lavage (BAL) fluid or lung biopsy specimens plus no evidence of small-vessel vasculitis (pulmonary capillaritis) or another explanatory diagnosis; it is confirmed by lung biopsy if other findings are inconclusive.

Treatment

  • Corticosteroids

Corticosteroids may reduce the morbidity and mortality of acute episodes of alveolar bleeding and may control the disease progression of pulmonary fibrosis. Some patients may require additional immunosuppressive drugs.

Patients with celiac disease should be on a gluten-free diet.