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.

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Journal
Journal of Respiratory Medicine
Published
2026-04-17
DOI
https://doi.org/10.17116/respmed2026201154
Primary Topic
Vasculitis and related conditions
Type
article
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article

Current concepts of idiopathic pulmonary hemosiderosis in children

Svetlana I. Petrova, A. A. Kuznetsova, O. G. Malyshev, D.Yu. Ovsyannikov
Journal of Respiratory Medicine
Vasculitis and related conditions
article

Current concepts of idiopathic pulmonary hemosiderosis in children

Svetlana I. Petrova, A. A. Kuznetsova, O. G. Malyshev, D.Yu. Ovsyannikov
article en

Abstract

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.

Journal of Respiratory MedicineVol. 2(1)
Peoples' Friendship University of Russia (RU), Saint Petersburg State Pediatric Medical University (RU)
Good health and well-being
Openalex Percentile: Top 15%
Vasculitis and related conditions
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Current concepts of idiopathic pulmonary hemosiderosis in children — Svetlana I. Petrova, A. A. Kuznetsova, et al. · Journal of Respiratory Medicine (2026) | TGRS Research Map | TGRS