Idiopathic pulmonary hemosiderosis

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Disease Overview

Idiopathic pulmonary hemosiderosis is a rare disease characterized by repeated episodes of bleeding into the lungs, which can cause anemia and lung disease. The body is able to remove most of the blood from the lungs, but a large amount of iron is left behind. Over time, this iron can cause permanent damage to the lungs (fibrosis). Symptoms can resemble pneumonia and include coughing, coughing up blood (hemoptysis), difficulty breathing, and wheezing.[3781] 

The cause of idiopathic pulmonary hemosiderosis is unknown. Diagnosis is based on ruling out other kinds of pulmonary hemosiderosis, and tests may include imaging, laboratory tests, and a lung biopsy.[3781] Treatment often includes corticosteroids or other immunosuppressive medications.[13410] The prognosis may vary depending on the amount of pulmonary bleeding and age of diagnosis.[3781] 


Synonyms

  • Hemosiderosis, pulmonary, with deficiency of gamma-a globulin
  • Alveolar hypoventilation syndrome
  • Pulmonary hemosiderosis

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National Organization for Rare Disorders