Idiopathic pulmonary haemosiderosis (IPH) is an uncommon form of pulmonary haemosiderosis. It is characterised by the triad of
- iron deficiency anemia
- diffuse pulmonary infiltrates, usually represented by diffuse pulmonary haemorrhage
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 recognised gender predilection.
Haemosiderin-laden macrophages 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 haemorrhage without associated glomerulonephritis or serological abnormality.
Can be variable dependent on the phase and extent of the disease. Can range from showing diffuse pulmonary haemorrhage to fibrotic changes in the context of repeated events.
Treatment and prognosis
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.
History and etymology
It was first described by Ceelen in 1921 2.
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