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Eur Respir J 2004; 24:162-169
Copyright ©ERS Journals Ltd 2004

Idiopathic pulmonary haemosiderosis revisited

O.C. Ioachimescu1, S. Sieber2 and A. Kotch3

1 Dept of Pulmonary Allergy and Critical Care Medicine, Cleveland Clinic foundation, Cleveland, OH, and 2 Dept of Pathology and 3 Dept of Pulmonary Medicine and Critical Care, Danbury Hospital, Yale School of Medicine, Danbury, CT, USA

CORRESPONDENCE: O.C. Ioachimescu, 9500 Euclid Ave A90, Cleveland OH, 44195, USA. Fax: 1 2164458160. E-mail: oioac@yahoo.com

Keywords: Azathioprine, diffuse alveolar haemorrhage, idiopathic pulmonary haemosiderosis, immunosuppressants, interstitial lung disease.

Received: December 13, 2002
Accepted February 10, 2004

Abstract

Idiopathic pulmonary haemosiderosis is a rare cause of diffuse alveolar haemorrhage of unknown aetiology. It occurs most frequently in children, has a variable natural history with repetitive episodes of diffuse alveolar haemorrhage, and has been reported to have a high mortality. Many patients develop iron deficiency anaemia secondary to deposition of haemosiderin iron in the alveoli.

Examination of sputum and bronchoalveolar lavage fluid can disclose haemosiderin-laden alveolar macrophages (siderophages), and the lung biopsy shows numerous siderophages in the alveoli, without any evidence of pulmonary vasculitis, nonspecific/granulomatous inflammation, or deposition of immunoglobulins. Contrary to earlier reports, corticosteroids alone or in combination with other immunosuppressive agents may be effective for either exacerbations or maintenance therapy of idiopathic pulmonary haemosiderosis.




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