系统性红斑狼疮并发腹膜后纤维化的临床特征分析  

Clinical analysis of the retroperitoneal fibrosis with systemic lupus erythematosus

作　　者：胡洪涛[1] 高月[1] 姚涛[1] 曾芳[1] 陈玲[1] 水华[1] 

机构地区：[1]武汉大学中南医院肾内科,武汉430071

出　　处：《临床肾脏病杂志》2016年第6期340-344,共5页Journal Of Clinical Nephrology

摘　　要：目的分析系统性红斑狼疮（systemic lupus erythematosus，SLE）并发腹膜后纤维化（retroperitoneal fibrosis，RPF）的发病机制、临床表现、实验室检查、影像学检查以及治疗预后，旨在提高临床医师对SLE并发腹膜后纤维化的认识。方法收集整理我科1例SLE并发RPF患者的临床资料并进行分析，同时查阅并复习国内外相关文献，总结SLE并发RPF的临床特征。结果本文报道1例及国外报道7例SLE并发RPF病例，其中6例为女性患者，2例为男性患者，发病年龄17-54岁，中位年龄为26岁，平均年龄（30．9±15．8）岁。8例SLE并发RPF患者中，4例为腰腹痛就诊，3例为水肿，1例为关节痛。实验室检查提示8例患者均出现不同程度的蛋白尿、血尿，除1例未提及，其他7例抗核抗体均阳性。8例患者中，1例因病情恶化而死亡，7例行糖皮质激素或激素联合外科手术，病情好转后出院，随访无复发。结论SLE并发RPF在临床上属于罕见病，目前发病机制不清，主要认为与自身免疫性疾病有关，临床症状无特异性，主要表现为局部疼痛和下肢水肿，其诊断主要依赖于影像学检查，目前治疗建议使用激素和免疫抑制剂，对于病变累及输尿管引起输尿管梗阻肾积水的患者可行外科输尿管松解术。Objective To analyze the pathogenesis, clinical presentation, imaging findings, therapy and outcome of systemic lupus erythematosus （SLE） patient with retroperitoneal fibrosis （RPF）, aiming to improve the understanding of clinicians on SLE in combination with RPF. Methods The details of one patient concurrently suffering from SLE combined with RPF in my de- partment were collected and analyzed, and 7 cases of SLE combined with RPF reported abroad were reviewed to summarize the clinical characteristics. Results Including this case, a total of 8 SLE patients combined with RPF were selected for review. Six out of 8 were women with the age ranging from 17 to 54 years old[mean 30. 5 years（30. 5 ± 14. 6）]. Four patients were admitted into hospital for complaint of abdominal pain or back pain, 3 for edema, and one for arthralgia. Laboratory abnormalities mainly included proteinuria, and hematuresis in all 8 cases. ANA was positive in 7 cases. Except one death of disseminated intravascular coagulation（DIC）, the rest 7 cases achieved improvement with no relapse after discharge. Conclusions SLE in combination with RPF, a rare disease with the mechanism obscure, is considered an autoimmune disorder at present. CT and MRI are the two tests of choice in diagnosis and follow-up. A combination of corticosteroids and immunosuppressive drugs such as azathioprine is usually applied. Laparoscopic ureterolysis, or robotic ureterolysis, if available; is the technique of choice to relive obstruction.

关 键 词：腹膜后纤维化 系统性红斑狼疮 蛋白尿 

分 类 号：R593.241[医药卫生—内科学]