Lung
Other nonneoplastic disease
Idiopathic pulmonary hemosiderosis
Author: Elliot Weisenberg, M.D.
Last author update: 1 December 2011
Last staff update: 30 September 2020
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed search: idiopathic pulmonary hemosiderosis [title]
Table of Contents
Definition / general | Microscopic (histologic) description | Differential diagnosisCite this page: Weisenberg E. Idiopathic pulmonary hemosiderosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungnontumoridiopulmhemo.html. Accessed December 4th, 2024.
Definition / general
- Rare, usually children or teenagers with hemoptysis, hypoxemia, dypsnea and iron deficiency anemia similar to Goodpasture’s disease, but no antibodies in serum or tissue, no renal disease
- Clinical triad of hemoptysis, iron deficiency and diffuse pulmonary infiltrates
- May be related to consanguinity or environmental factors
- Fever, lymph node, liver and spleen enlargement may occur
- Usually fatal after several years
- Diagnosis of exclusion
Microscopic (histologic) description
- Hemosiderin laden macrophages in alveolar lumina, shedding and hyperplasia of alveolar epithelial cells and marked alveolar capillary congestion
- Varying interstitial fibrosis, intra-alveolar hemorrhage
- No necrosis, no vasculitis, no granulomas and no lymphoid follicles
Differential diagnosis
- Note: these disorders all cause secondary pulmonary alveolar bleeding and hemosiderosis
- Mitral stenosis
- Periarteritis nodosa
- SLE (neutrophils in alveolar septal capillaries)
- Systemic vasculitis
