Prognosis of chinese children with idiopathic pulmonary hemosiderosis after long-term maintenance glucocorticoid therapy

Abstract

Abstract Objective To explore the prognosis of children with idiopathic pulmonary hemosiderosis (IPH) after long-term maintenance of glucocorticoid therapy. Methods This ambidirectional observational study included children with IPH admitted to the Department of Respiratory, Beijing Children’s Hospital, Capital Medical University, between January 2010 and July 2021. Their clinical characteristics were collected from medical records and during follow-up. Results A total of 211 children (88 males,123 females, age of onset: 3.42 [0.33–12.75] years, the median time from onset to diagnosis: 2.8 months [4 days-8 years]) with IPH were included. All children received glucocorticoid therapy in the acute alveolar hemorrhage phase. After discharge, 5 (2.4%) children lost to follow-up, 11 (5.2%) died during follow-up, and a total of 195 patients survived, with a median follow-up time of 5.52 (1.43, 12.58) years, the 5-year and 10-year survival rate were 95.9% and 86.2%, respectively. Of the 195 patients, Clinical remission was achieved in 65 patients (33.3%), including 61 patients treated with glucocorticoid alone and 4 patients treated with immunosuppressive agents. 109 (55.9%) were in a stable phase with medications, and 21 (10.8%) had no clinical remission. Of the 195 patients who survived, 141(72.3%) were treated with glucocorticoid alone and 54(26.7%) with immunosuppressive agents. Four (2.05%) patients developed secondary cataracts, and 33 (16.92%) patients were short in stature. Conclusion Long-term low-dose glucocorticoid maintenance therapy might improve the prognosis of IPH children, but the side effects should be monitored.