ArticleOrphanet journal of rare diseases2015
A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis.
Article in Orphanet journal of rare diseases, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 42 citations in OpenAlex.
- Case Report: Effective use of rituximab in children with steroid-resistant/dependent idiopathic pulmonary hemosiderosis: description of two cases and literature review.Frontiers in pediatrics · 2026Article
- Idiopathic Pulmonary Haemorrhage, Is It Really Idiopathic?Respirology case reports · 2025Article
- Neuropsychiatric Side Effects of Hydroxychloroquine in a Patient With Idiopathic Pulmonary Hemosiderosis.Journal of paediatrics and child health · 2025Article
- Clinical features and risk factors for recurrence of idiopathic pulmonary hemosiderosis in children.BMC pulmonary medicine · 2024Observational
- Idiopathic pulmonary haemosiderosis misdiagnosed as haemolytic anaemia: a case report.The Journal of international medical research · 2023Article
- Lane-Hamilton syndrome.Respirology case reports · 2023Article
- Idiopathic pulmonary hemosiderosis and stroke secondary to protein C deficiency in a child with Down syndrome: a case report.Journal of medical case reports · 2023Article
- Diffuse alveolar haemorrhage in children: an international multicentre study.ERJ open research · 2023Article
- Diffuse alveolar haemorrhage in children hospitalised in a tertiary‑level hospital: A retrospective descriptive study.African journal of thoracic and critical care medicine · 2023Article
- Adult patients with idiopathic pulmonary hemosiderosis: a comprehensive review of the literature.Clinical rheumatology · 2022Review
- Proposed Pathogenesis of Diffuse Alveolar Hemorrhage in Idiopathic Pulmonary Hemosiderosis.Lung · 2022Review
- Review
- Differentiation of idiopathic pulmonary hemosiderosis from rheumatologic and autoimmune diseases causing diffuse alveolar hemorrhage: establishing a diagnostic approach.Clinical rheumatology · 2022Review
- Diffuse alveolar haemorrhage with suspected idiopathic pulmonary hemosiderosis and decrease in lung diffusing capacity and chronic respiratory failure.BMJ case reports · 2021Article
- Idiopathic pulmonary hemosiderosis: a review of the treatments used during the past 30 years and future directions.Clinical rheumatology · 2021Review
- Idiopathic Pulmonary Hemosiderosis as a Mimic of Pulmonary Vasculitis: A Case Report and Review of the Literature.Current allergy and asthma reports · 2020Review
- Disease recurrence after lung transplantation for idiopathic pulmonary hemosiderosis.Respiratory medicine case reports · 2020Article
- [Diffuse alveolar hemorrhage in children].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2019Review
- Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients.The Journal of international medical research · 2019Article
- Pulmonary Hemorrhage in Children: Etiology, Clinical Profile and Outcome.Indian journal of pediatrics · 2019Article
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIdiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology characterized by chronic pulmonary hemorrhage and presents with a triad of anemia, hemoptysis and pulmonary infiltrates. IPH is a diagnosis of exclusion with a variable and disparate clinical course. Despite existing therapies, few children achieve full remission while others have recurrent hemorrhage, progressive lung damage, and premature death.
methodsWe surveyed physicians who care for patients with IPH via a web-based survey to assess the most common practices. 88 providers responded, caring for 274 IPH patients from five continents.
results63.3 % of respondents had patients that were initially misdiagnosed with anemia (60.0 %) or gastrointestinal bleed (18.2 %). Respondents varied in diagnostic tools used for evaluation. The key difference was in the use of lung biopsy (51.9 %) for diagnosis. Common medications respondents used for treatment at initial presentation and chronic maintenance therapy were corticosteroids (98.7 and 84.0 %, initial and chronic therapy respectively), hydroxychloroquine (33.3 and 64.0 %), azathioprine (8.0 and 37.3 %), and cyclophosphamide (4.0 and 16.0 %). There was agreement on the use of corticosteroids for exacerbation amongst all respondents. Reported deaths before adulthood occurred in 7.3 % of patients. We conclude that there were common features and specific variations in physician management of IPH. Respondents were divided on whether to perform lung biopsy for diagnosis.
conclusionDespite the availability of various immunomodulators, corticosteroids remained the primary therapy. We speculate that the standardization of care for diffuse alveolar hemorrhage will improve patient outcomes.
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