Lung - nontumor
Other nonneoplastic disease
Idiopathic pulmonary hemosiderosis

Author: Elliot Weisenberg, M.D. (see Authors page)

Revised: 9 March 2017, last major update December 2011

Copyright: (c) 2003-2017,, Inc.

PubMed search: idiopathic pulmonary hemosiderosis [title]

Cite this page: Idiopathic pulmonary hemosiderosis. website. Accessed September 25th, 2018.
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