Sasaki Emi, Shibata Maki, Kato Asami, Hamano Naoto, Katsuki Takashi, Tada Manami, Hinoshita Fumihiko
Department of Nephrology, National Center for Global Health and Medicine Hospital, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan.
Tokyo Medical Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-0034, Japan.
CEN Case Rep. 2016 Nov;5(2):212-218. doi: 10.1007/s13730-016-0227-0. Epub 2016 Jul 13.
A previously healthy 37-year-old Canadian man living in Japan visited a hospital in Thailand while traveling because of edematous legs, purpura, arthralgia, bloody stool, and fever after an insect bite. Henoch-Schönlein purpura (HSP) was suspected. His creatinine level was 5.2 mg/dL. He was treated with oral prednisolone (PSL) and oral cyclophosphamide (CPA); after treatment, his creatinine level improved to 2.4 mg/dL. Upon returning to Japan, he was admitted to the National Center for Global Health and Medicine Hospital in Tokyo. A kidney biopsy was performed, and HSP nephritis (HSPN) was diagnosed. Renal dysfunction and proteinuria persisted despite 4 administrations of steroid-pulse therapy and 3 sessions of plasma exchange. Finally, he was treated with intravenous cyclophosphamide (IVCY). His creatinine level and proteinuria markedly improved. His microscopic hematuria disappeared after he underwent tonsillectomy. There have been only a few case reports describing patients with adult-onset HSPN necessitating IVCY. We present here a rare case of steroid-resistant HSPN treated with IVCY and tonsillectomy, with reference to some recent findings.
一名居住在日本的37岁既往健康的加拿大男子在旅行期间因腿部水肿、紫癜、关节痛、便血和昆虫叮咬后发热前往泰国一家医院就诊。怀疑为过敏性紫癜（HSP）。他的肌酐水平为5.2mg/dL。给予口服泼尼松龙（PSL）和口服环磷酰胺（CPA）治疗；治疗后，他的肌酐水平改善至2.4mg/dL。回到日本后，他入住了东京的国立全球健康与医学中心医院。进行了肾活检，诊断为HSP肾炎（HSPN）。尽管进行了4次类固醇冲击治疗和3次血浆置换，肾功能不全和蛋白尿仍持续存在。最后，给予他静脉注射环磷酰胺（IVCY）治疗。他的肌酐水平和蛋白尿明显改善。他在接受扁桃体切除术后镜下血尿消失。仅有少数病例报告描述了需要IVCY治疗的成人起病HSPN患者。我们在此介绍一例罕见的对类固醇耐药的HSPN病例，该病例采用IVCY和扁桃体切除术治疗，并参考了一些最新研究结果。
