Clinical Observation And Analysis Of Long-term Use Of Corticosteroids In The Management Of Idiopathic Pulmonary Haemosiderosis

Background: Idiopathic pulmonary hemosiderosis(IPH)is a rare chronic disorder,characterized by recurrent pulmonary hemorrhage.Common clinical features include iron-deficiency anaemia which responds to treatment with iron but liable to relaps,and respiratory symptoms such as cough,hemoptysis,wheezing,dyspnea.There is no evidence-based guidelines for treatment.First line treatment usually include systemic corticosteroid therapy,which could reduce the morbidity and mortality of acute episodes of alveolar bleeding.Long-term use of corticosteroids.can be problematic because of well-known side effects,and that may have no effect on the overall course or delaying pulmonary fibrosis.Objective:Analyzing the IPH patients followed up in our department,discussing the treatment process,efficacy,side effects and prognosis of Long-term of corticosteroids in children with IPH.Methods:A retrospective review of 62 cases of IPH followed up in our department during 2004-2017.The duration of disease is 1-13 years,with corticosteroids as the main treatment.Of 62 children,11 lost to visit,10 died and 41 survived.Analyzing the tapering course of steroids,lesions on chest CT,lung function,bone density,height,weight etc.of the 41 survived cases,and discussing 8 cases of children entered ICU or died.Conclusion:Pulmonary hemorrhage can be relieved by the treatment of corticosteroids with pulse therapy or non-pulse therapy.It could relapse after reduction or withdrawal of corticosteroids,then increase the dose of corticosteroids could control the hemorrhage.While different degree of interstitial pulmonary fibrosis leaves on chest CT after recurrent pulmonary hemorrhage,there is no obvious abnormality in activities of daily living and basic lung function.Side effects of corticosteroids such as short stature,bone loss and Cushing’s appearance can not be ignored.The occurrence of short stature is also related to the disease itself.After periods of remission,sudden hemorrhagic flares may occur unpredictably,usually deteriorating in 1-2 days and needs immediate rescue.