Aggressive corticosteroid treatment in childhood idiopathic pulmonary hemosiderosis with better outcome

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare but fatal disease characterized by a triad of anemia, hemoptysis, and increased pulmonary infiltration. This study is aimed to review the clinical manifestations, diagnostic tools, medication and outcome of childhood IPH in Taiwan. We retrospectively enrolled the patients less than 18 years old in National Taiwan University Hospital in the past 30 years. The clinical data were collected and analyzed. All of the twelve children diagnosed with IPH had anemia and increased pulmonary infiltration, eight had hemoptysis, and ten were confirmed with detection of hemosiderin-laden macrophages. The mean age at diagnosis were 4.9 (interquartile range 2.5-6.3) years old. Patients with high dose corticosteroid (CS, ≥ 1mg/kg/day prednisolone equivalent) treatment had lower odds ratio for ICU admission and significant higher Hb recovery rate than those with mild disease activity not receiving high dose CS treatment (p=0.011). The only factor that is significantly associated with persistent anemia is the usage of high dose CS (p<0.001) after adjusting for hemoptysis, fulfilling triad, serum ferritin level, and ICU admission by multiple regression. The only factor that is significantly associated with ICU admission is the presence of microorganism yielded in sputum (p<0.001) after adjusting for fever, serum ferritin level, usage of invasive MV, and high dose CS treatment days. The aggressive high dose CS therapy might prevent ICU admission and improve anemia. Aggressive high dose CS treatment is suggested in IPH patients regardless of the disease activity.