26例IgG4相关性腹膜后纤维化的系统评价

Systematic review of 26 IgG4-related retroperitoneal fibrosis in 26 cases

目的 探讨IgG4相关性腹膜后纤维化的临床病理特点、治疗及预后情况.方法 联机检索中国期刊全文数据库、维普中文科技期刊数据库、Medlme/Pubmed及OVID全文数据库,检索截止至2013年11月,结合南京大学医学院附属鼓楼医院消化科诊治的1例患者,进行系统评价.结果 15篇文献进入本系统综述,共纳入26例诊断明确的病例.系统评价结果显示（1）该病好发于69岁左右男性,临床表现以腰背痛、腹痛及肾盂积水为主;（2）血清IgG4升高多见;CT或MRI提示腹膜后肿块;（3）组织病理学检查显示软组织纤维化和IgG4＋浆细胞聚集;（4）目前无标准化方案,糖皮质激素仍是治疗IgG4相关性腹膜后纤维化的首选方案.结论 IgG4相关性腹膜后纤维化是一新的疾病亚型,临床症状缺乏特异性,极易误诊,需进一步研究以提高对该病的认识.

Objective To investigate the clinical pathologic features, therapy, and prognosis of IgG4-related retrop- eritoneal fibrosis. Methods Databases including Chinese Journal Full-text Database, VIP Database for Chinese Technical Periodicals, Medline/Pubmed, and OVID were retrieved electronically up to November 2013. A systematic review was performed together with one case in our hospital. Results 26 patients in fifteen literatures fulfilling the pathological criteria were confirmed the diagnosis of IgG4-related retroperitoneal fibrosis. The results of this systematic review showed that： （1） IgG4-related retroperitoneal fibrosis was more common in men at the median age of 69. The most common presentations were back pain, abdominal pain, and hydronephrosis. （2） Most cases were found to have abnormally elevated serum IgG4 levels. CT or MRI scans showed retroperitoneal masses. （ 3）Histological examination showed soft tissue fibrosis accompanied by IgG4-positive plasma cells aggregates. （4） There was no consensus on the treatment of IgG4-related retroperitoneal fib- rosis. Steroid therapy was currently considered as the first line treatment for these series of patients. Conclusion IgG4- related retroperitoneal fibrosis is a new subtype of IgG4-related disease. It has no specific character, and is easily to be misdiagnosed. Further research is necessary to improve the comprehensive understanding of this disease.