Idiopathic pulmonary hemosiderosis (IPH) is an uncommon form of pulmonary hemosiderosis. It is characterized by the triad of
- iron deficiency anemia
- diffuse pulmonary infiltrates, usually represented by diffuse pulmonary hemorrhage
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.
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.
- 1. Castañer E, Alguersuari A, Gallardo X et-al. When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics. 2010;30 (1): 33-53. doi:10.1148/rg.301095103 - Pubmed citation
- 2. Fleischner FG, Berenberg. Idiopathic pulmonary hemosiderosis. Radiology. 1954;62 (4): 522-6. doi:10.1148/62.4.522 - Pubmed citation
- 3. Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur. Respir. J. 2005;24 (1): 162-70. Pubmed citation
- 4. Yao TC, Hung IJ, Jaing TH et-al. Pitfalls in the diagnosis of idiopathic pulmonary haemosiderosis. Arch. Dis. Child. 2002;86 (6): 436-8. Free text at pubmed - Pubmed citation
- 5. Keller C, Langston C. Images in clinical medicine. Childhood idiopathic pulmonary hemosiderosis. N. Engl. J. Med. 2000;343 (11): 781. doi:10.1056/NEJM200009143431105 - Pubmed citation