Current concepts of idiopathic pulmonary hemosiderosis in children
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease associated with recurrent diffuse alveolar hemorrhage and progressive accumulation of hemosiderin in the lung parenchyma. The classical clinical presentation is characterized by a triad: hemoptysis, iron-deficiency anemia, and diffuse pulmonary infiltrates. The incidence of IPH is estimated at 0.24—1.23 per 1 million children per year, and the disease course may be severe and accompanied by considerable mortality, underscoring the importance of timely diagnosis. This article systematizes current data on epidemiology and possible mechanisms of disease development, summarizes diagnostic approaches to the identification of diffuse alveolar hemorrhage, and reviews key methods for diagnostic confirmation, including bronchoscopy with bronchoalveolar lavage and morphological examination of lung tissue. Particular attention is given to differential diagnosis and contemporary therapeutic strategies based on systemic glucocorticosteroids and immunosuppressive therapy, which may reduce the frequency of relapses and improve disease outcomes.
Authors
- Svetlana I. Petrova (ORCID: https://orcid.org/0000-0003-4212-416X)
- A. A. Kuznetsova
- O. G. Malyshev
- D.Yu. Ovsyannikov (ORCID: https://orcid.org/0000-0002-4961-384X)
Institutions
- Peoples' Friendship University of Russia (RU)
- Saint Petersburg State Pediatric Medical University (RU)
Publication Details
- Journal
- Journal of Respiratory Medicine
- Published
- 2026-04-17
- DOI
- https://doi.org/10.17116/respmed2026201154
- Primary Topic
- Vasculitis and related conditions
- Type
- article
- Field-Weighted Citation Impact
- 0.00