Frontiers in pediatrics · 2026
Case Report: Effective use of rituximab in children with steroid-resistant/dependent idiopathic pulmonary hemosiderosis: description of two cases and literature review.
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Li Sha, Liu Tinghua, Ou Rongqiong, Wang Haiyan, Zhang Bihong, Tan Weiping
Аннотация
INTRODUCTION: Idiopathic pulmonary hemosiderosis (IPH) is a rare, chronic, and potentially life-threatening disorder predominantly affecting the pediatric population. While most patients respond to conventional immunosuppressive therapy, management of steroid-resistant/dependent cases remains challenging. This study aims to evaluate the efficacy and safety of rituximab (RTX) in the treatment of steroid-resistant/dependent IPH in children.
METHODS: We report two pediatric cases of steroid-resistant/dependent IPH who were admitted to our institution. Both patients presented with severe, recurrent diffuse pulmonary hemorrhage and demonstrated poor response to conventional therapy including high-dose corticosteroid pulse therapy and combination immunosuppressants. Clinical data, laboratory findings, radiographic features, treatment regimens, and outcomes were collected and analyzed.
RESULTS: Both patients exhibited steroid resistance or dependence with persistent or recurrent pulmonary hemorrhage despite aggressive conventional immunosuppression. Following RTX administration, rapid and sustained hemorrhage control was achieved in both cases. Clinical remission was maintained over follow-up periods of 6 months for both patients, with successful corticosteroid tapering and no significant infusion-related adverse events observed. No disease relapse occurred during the follow-up period.
CONCLUSION: Rituximab appears to be an effective and well-tolerated rescue therapy for steroid-resistant/dependent IPH in children, offering sustained hemorrhage control and facilitating corticosteroid-sparing. Given the limited existing literature on RTX application in IPH management, this report offers a novel therapeutic approach for steroid-resistant/dependent IPH cases.
METHODS: We report two pediatric cases of steroid-resistant/dependent IPH who were admitted to our institution. Both patients presented with severe, recurrent diffuse pulmonary hemorrhage and demonstrated poor response to conventional therapy including high-dose corticosteroid pulse therapy and combination immunosuppressants. Clinical data, laboratory findings, radiographic features, treatment regimens, and outcomes were collected and analyzed.
RESULTS: Both patients exhibited steroid resistance or dependence with persistent or recurrent pulmonary hemorrhage despite aggressive conventional immunosuppression. Following RTX administration, rapid and sustained hemorrhage control was achieved in both cases. Clinical remission was maintained over follow-up periods of 6 months for both patients, with successful corticosteroid tapering and no significant infusion-related adverse events observed. No disease relapse occurred during the follow-up period.
CONCLUSION: Rituximab appears to be an effective and well-tolerated rescue therapy for steroid-resistant/dependent IPH in children, offering sustained hemorrhage control and facilitating corticosteroid-sparing. Given the limited existing literature on RTX application in IPH management, this report offers a novel therapeutic approach for steroid-resistant/dependent IPH cases.