Idiopathic pulmonary haemosiderosis

Idiopathic pulmonary haemosiderosis (IPH) is an uncommon form of pulmonary haemosiderosis. It is characterised 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 recognised gender predilection.

Haemosiderin-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 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.

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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Article information

rID: 40763
System: Chest
Tag: cases
Synonyms or Alternate Spellings:
  • Idiopathic pulmonary hemosiderosis
  • Idiopathic pulmonary haemosiderosis (IPH)

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