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Updated: Aug 18, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Idiopathic primary pulmonary hemosiderosis: treatment with cyclophosphamide and prednisone]
S E Viera1, A A Osmo, J P Lotufo
1Divisão de Pediatria, Hospital Universitario da USP, São Paulo, SP.
Insights
Idiopathic pulmonary hemosiderosis in a child showed poor response to prednisone and chloroquine. Adding cyclophosphamide led to remission, suggesting this combination may benefit selected cases.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Immunosuppressive Therapy
Context:
- Idiopathic pulmonary hemosiderosis (IPH) is a rare condition causing lung bleeding.
- Pediatric IPH cases often present diagnostic and therapeutic challenges.
Purpose:
- To report a pediatric case of idiopathic pulmonary hemosiderosis.
- To evaluate the efficacy of a treatment regimen including cyclophosphamide.
Summary:
- A five-year-old child with IPH initially responded poorly to prednisone and chloroquine.
- Combination therapy with prednisone and cyclophosphamide induced clinical and radiological remission.
- The patient remained in remission for one year after discontinuing medication.
Impact:
- This case suggests cyclophosphamide and prednisone may be a viable treatment for selected pediatric IPH patients.
- Highlights the potential for successful management of refractory IPH with immunosuppressive agents.
Abstract:
The authors present the case of a child diagnosed as having idiopathic pulmonary hemosiderosis at five years of age who had a good clinical outcome at the age of ten years. Initially the patient was treated with prednisone and chloroquine with poor results. When cyclophosphamide was added to prednisone, the patient demonstrated clinical and radiological remission. To date, the patient has been followed for one year without any medication, and has had only one limited episode of hemosiderosis. The authors also suggest that the therapeutic regimen with cyclophosphamide and prednisone may be useful for some selected cases.
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