Idiopathic pulmonary hemosiderosis

Last revised by Daniel J Bell on 13 Jul 2021

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon form of pulmonary hemosiderosis. It is characterized by the triad of

The diagnosis is usually made by exclusion 1.

It is rare disorder that can occur at any age but typically affects children and young adults. The age of presentation may be bimodal, with frequency peaks in children less than five years of age and in adolescents 11 years or older. There is no definite recognized gender predilection.

Hemosiderin-laden macrophages are found in the sputum or at bronchoalveolar lavage. Its exact cause is unknown but may be related to an autoimmune process. Patients have recurrent episodes of diffuse pulmonary hemorrhage without associated glomerulonephritis or serological abnormality.

Can be variable dependent on the phase and extent of the disease. Can range from showing diffuse pulmonary hemorrhage to fibrotic changes in the context of repeated events.

It can have a variable natural history 3. Some authors suggest corticosteroid (alone or in combination with other immunosuppressive agents) for exacerbations or maintenance therapy. 

It was first described by Ceelen in 1921 2.

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