Lung

Other nonneoplastic disease

Idiopathic pulmonary hemosiderosis



Topic Completed: 1 December 2011

Minor changes: 30 September 2020

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PubMed search: idiopathic pulmonary hemosiderosis [title]

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Idiopathic pulmonary hemosiderosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungnontumoridiopulmhemo.html. Accessed October 25th, 2021.
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
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