强直性脊柱炎继发淀粉样变三例并文献复习  

Amyloidosis secondary to ankylosing spondylitis: case report and literature review

作　　者：韩欣欣[1] 陈雨 邓垂文 彭琳一 曾学军[1] 张文 曾小峰 张奉春 Han Xinxin;Chen Yu;Deng Chuiwen;Peng Linyi;Zeng Xuejun;Zhang Wen;Zeng Xiaofeng;Zhang Fengchun(Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China;Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院普通内科,100730 [2]中国医学科学院北京协和医学院北京协和医院免疫内科,100730

出　　处：《中华全科医师杂志》2019年第3期267-270,共4页Chinese Journal of General Practitioners

摘　　要：为探讨强直性脊柱炎(AS)继发淀粉样变的临床特征、治疗和预后,回顾性分析北京协和医院确诊的3例AS合并淀粉样变患者的临床资料并复习相关文献。3例患者均存在肾脏受累,2例合并心脏受累,1例合并胃肠道受累,1例合并皮肤苔藓样变。检索1997—2017年万方数据库、Pubmed数据库相关文献,共检索到11例AS合并淀粉样变患者。14例患者中,男∶女比例为5∶2,诊断AS年龄(29.6±10.5)岁,确诊淀粉样变的年龄为(44.0±10.5)岁。8例表现为水肿、大量蛋白尿,2例腹泻,2例呼吸困难,2例无明显症状。13例肾脏受累,1例仅有心脏受累。13例肾脏受累的患者中,合并心脏受累2例,胃肠道受累2例,皮肤受累2例。发病时ESR(83.7±22.6)mm/1 h,CRP(89±59)mg/L。确诊淀粉样变后,9例患者用肿瘤坏死因子抑制剂治疗,3例对症治疗,1例行肾移植,1例腹膜透析。使用肿瘤坏死因子抑制剂的9例患者中,8例症状缓解,1例失访。对症治疗的3例患者死亡,肾移植及腹膜透析患者病情稳定。提示AS继发淀粉样变常发生于炎症控制不佳、长病程的青年男性,肾脏为最常见的受累脏器,应积极控制AS病情,部分报道肿瘤坏死因子抑制剂展现良好应用效果。Three patients diagnosed as amyloidosis secondary to ankylosing spondylitis (AS) in Peking Union Medical College Hospital are reported. All 3 cases had renal abnormalities, 2 cases were complicated with cardiac abnormalities, 1 case was complicated with gastrointestinal abnormalities and 1 case was complicated with skin moss-like changes. Eleven cases of AS with amyloidosis were retrieved from Wanfang database and Pubmed database from 1997 to 2017. In total 14 cases, the male and female ratio was 5∶2, the mean age of AS onset was (29.6±10.5) years, and the mean age of amyloidosis diagnosed was (44.0±10.5) years. Eight patients presented with edema and severe proteinuria, 2 cases showed diarrhea, 2 cases showed dyspnea, while 2 cases had no obvious symptoms. Thirteen cases had renal abnormalities and 1 case had only heart involvement. Of the 13 patients with renal involvement, 2 cases were complicated with cardiac abnormalities, 2 cases complicated with gastrointestinal abnormalities, 2 cases complicated with skin abnormalities.The mean ESR was (83.7±22.6) mm/1 h,C reactive protein was (89±59) mg/L at onset. After the diagnosis of amyloidosis, 9 patients were treated with TNF-α inhibitors, 3 cases received symptomatic therapy, 1 case underwent renal transplantation and 1 case were treated with peritoneal dialysis. In 9 patients treated with TNF-α inhibitors, 8 cases were relieved and 1 case lost follow-up. Three patients who received symptomatic treatment all died, while patients receiving kidney transplantation and peritoneal dialysis were in stable condition. The study indicates that secondary amyloidosis often occurs in young male AS patients who have long course and poorly controlled disease, the kidney is the most common affected organ. TNF-α inhibitors may be effective for treatment of amyloidosis secondary to AS.

关 键 词：脊柱炎 强直性 淀粉样变性 

分 类 号：R593.23[医药卫生—内科学]