Merck Manual

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Idiopathic Pulmonary Hemosiderosis

By

Joyce Lee

, MD, MAS, University of Colorado School of Medicine

Reviewed/Revised Oct 2023
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Idiopathic pulmonary hemosiderosis is a rare disease that causes recurrent diffuse alveolar hemorrhage Diffuse Alveolar Hemorrhage Diffuse alveolar hemorrhage is persistent or recurrent pulmonary hemorrhage that originates from the lung parenchyma (ie, the alveoli) as opposed to the airways. There are numerous causes, but... read more Diffuse Alveolar Hemorrhage with no detectable underlying disorder; it occurs mainly in children < 10 years. In patients with idiopathic pulmonary hemosiderosis, repeated alveolar bleeding can eventually result in pulmonary hemosiderosis and fibrosis.

Symptoms and Signs of Idiopathic Pulmonary Hemosiderosis

Diagnosis of Idiopathic Pulmonary Hemosiderosis

  • 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.

Diagnosis is confirmed by lung biopsy if other findings are inconclusive.

Treatment of Idiopathic Pulmonary Hemosiderosis

  • 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 immunosuppressants.

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

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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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