Association of Bronchial Artery Embolization with Disease Recurrence in paediatric Idiopathic Pulmonary Hemosiderosis: A Retrospective Cohort Study

OBJECTIVE This study aimed to characterize pediatric idiopathic pulmonary hemosiderosis (IPH) and assess whether adding bronchial artery embolization (BAE) to glucocorticoid (GC) therapy reduces the cumulative risk of disease recurrence. METHODS We conducted a retrospective cohort study of children with IPH between January 2003 and January 2020 from a single center. Demographic, clinicopathologic, therapeutic, and outcomes data were extracted. The primary outcome was cumulative recurrence of hemorrhage, analysed using the Andersen-Gill models. The secondary outcomes included time to first recurrence and change in hemoglobin levels from baseline to six months after treatment. RESULTS A total of 63 patients were included, with 44 (70%) receiving BAE plus GC treatment and 19 (30%) receiving GC treatment alone. The median age at diagnosis was 4.6 years (interquartile range: 2.1–7.6), and 28 (44%) were female. During a median follow-up period of 36 months (range, 0.7–120), 87 relapses occurred in the GC with BAE group versus 24 in the GC without BAE group. The adjusted hazard ratio was 1.66 (95% confidence interval [CI], 0.58–4.74) for the GC with BAE group compared with GC alone. The corresponding hazard ratio for time to first recurrence was 0.98 (95% CI, 0.39–2.44). Changes in hemoglobin levels did not differ significantly between the two groups (–20.5 [95% CI, − 27.4 to − 0.7] vs − 14.0 [95% CI, − 26.7 to 14.4], mean difference − 6.5 [95% CI, − 19.0 to 6.0], P  = 0.30). CONCLUSIONS Our data do not support the addition of BAE to conventional GC therapy as routine primary treatment for pediatric IPH.