Idiopathic pulmonary haemosiderosis revisited. Eur Respir J 2004 Jul;24(1):162-70
Date
08/06/2004Pubmed ID
15293620DOI
10.1183/09031936.04.00116302Scopus ID
2-s2.0-3543098723 (requires institutional sign-in at Scopus site) 170 CitationsAbstract
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.
Author List
Ioachimescu OC, Sieber S, Kotch AAuthor
Octavian C. Ioachimescu MD, PhD Vice Chair, Director, Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultBiopsy, Needle
Blood Chemical Analysis
Female
Hemosiderosis
Humans
Immunohistochemistry
Immunosuppressive Agents
Incidence
Lung
Lung Diseases
Male
Prognosis
Radiography, Thoracic
Respiratory Function Tests
Risk Assessment
Severity of Illness Index
Survival Rate
Tomography, X-Ray Computed
Treatment Outcome
