Rituximab as a Steroid-Sparing Therapy in Pediatric Idiopathic Pulmonary Hemosiderosis: A Case Report.

OBJECTIVE

Idiopathic pulmonary hemosiderosis (IPH) is a rare pediatric disorder characterized by recurrent alveolar hemorrhage leading to iron-deficiency anemia and diffuse pulmonary infiltrates. Its diagnosis is frequently delayed because early manifestations often mimic common respiratory or hematologic conditions. High-dose corticosteroids remain the mainstay of acute management, yet prolonged steroid dependence is associated with significant sequelae. Emerging evidence suggests that B cell-directed therapy, including rituximab, may offer a steroid-sparing alternative in refractory pediatric IPH.

CLINICAL PRESENTATION AND INTERVENTION: We describe an uncommon case of steroid-dependent IPH. Her clinical course was marked by recurrent hypoxemic episodes, diffuse alveolar bleeding, and severe anemia, leading to multiple admissions to intensive care and repeated transfusion requirements. Although she received prolonged therapy with corticosteroids, hydroxychloroquine, and azathioprine, she consistently relapsed whenever steroid doses were reduced. Rituximab was introduced as a steroid-sparing strategy, after which she demonstrated significant clinical improvement and durable remission.

CONCLUSION

This case illustrates the diagnostic challenges of pediatric IPH when early features are incomplete and underscores the potential value of Rituximab as a steroid-sparing option in refractory disease. The patient's sustained remission following B cell-directed therapy supports its emerging role in IPH, though larger, well-designed studies are needed to better define its long-term safety and optimal use in children.