关键词: 肺含铁血黄素沉着症, 激素, 硫唑嘌呤, 羟氯喹, 儿童

Abstract: Objective  To increase the diagnosis and treatment level of idiopathic pulmonary hemosiderosis.
Methods  The clinical data of 19 children diagnosed with idiopathic pulmonary hemosiderosis from January 2013 to October 2019 were analyzed.
Results  Among the seven cases treated with hormone alone, three cases had no relapse, and the other four cases relapsed during hormone reduction. Seven cases of children treated with hormone combined with azathioprine relapsed repeatedly during the period of hormone treatment alone, five of which had no relapse after adding azathioprine (in 1 case azathioprine was used for only one month, and the parents stopped using azathioprine by themselves), and two cases relapsed once after adding azathioprine. In addition, there were three cases of repeated attacks during hormone therapy alone, and then azathioprine was used, but in two cases it was stopped because of the obvious decrease of granulocytes, and in one case it was stopped because of the serious vomiting and diarrhea reaction; the three cases were all treated with hormone combined with hydroxychloroquine, and the number of relapses and side effects were reduced after adding hydroxychloroquine. In the remaining two cases, one patient was diagnosed with juvenile idiopathic arthritis and Sjogren's syndrome one year after the diagnosis of IPH; in the treatment, hormone combined with methotrexate was used to control the disease and reduce the recurrence; the other patient was diagnosed with nephritis syndrome because of hematuria and proteinuria. He was treated with hormone combined with tacrolimus for one year. He still had hemoptysis and hematuria and proteinuria. After that, he was treated with hormone combined with leflunomide for more than half a year in another hospital. He had no attack of hemoptysis or hematuria or proteinuria.
Conclusion  For children diagnosed with IPH, if hormone therapy alone or hormone combined with azathioprine is not effective or if the patient 〖JP2〗can not tolerate side effects, hormone combined with hydroxychloroquine can be considered for treatment

Key words: Pulmonary hemosiderosis, Hormone, Azathioprine, Hydroxychloroquine, Children