特发性腹膜后纤维化16例临床分析

Clinical features of 16 patients with idiopathic retroperitoneal fibrosis

目的 探讨特发性腹膜后纤维化（iRPF）临床特点、诊疗及转归,以提高临床诊断率,减少漏诊和误诊.方法 回顾分析在青岛大学附属医院住院确诊的16例iRPF患者,其中包括9例IgG4相关腹膜后纤维化患者.结果 iRPF多见于中老年男性,肾功能衰竭合并腰腹疼痛、体重减轻是最常见的临床表现.IgG4-RPF患者与非IgG4-RPF患者Hb、SCr、ESR、CRP组间比较差异无统计学意义.所有患者均存在CT或MRI下腹膜后软组织密度影;IgG4-RPF患者同时还可发现颌下腺、深部淋巴结、胰腺、胆管、胸腺、肾间质、甲状腺等多器官受累.病理学检查发现全部IgG4-RD患者不同受累器官均显示相同特点的改变,且其IgG4阳性的浆细胞计数、IgG4 ＋/IgG阳性浆细胞计数比例、席纹状纤维化例数较非IgG4-RPF患者有显著的统计学差异.8例行皮质类固醇和免疫抑制剂治疗;6例行药物及手术联合治疗;2例单纯手术治疗.所有接受皮质类固醇治疗的患者病情均缓解,其中2例停药后复发;但单纯外科治疗患者反复发作.结论 特发性腹膜后纤维化与IgG4-RD疾病谱有交集,但临床特点及普通影像学检查无法做出鉴别,需要进一步病理学检查.激素及免疫抑制剂对两者治疗均有效并可减少复发.

Objectives The aim of this investigation is to explore clinical features,diagnosis,treatment and sequelae of idiopathic retroperitoneal fibresis （iRPF）,and to serve for improvement of clinical diagnosis and treatment.Methods Retrospective analysis were done in 16 cases with iRPF in the affiliated hospital of Qingdao University,including 9 patients with IgG4-related RPF.Results iRPF was more common in middle-aged and old males.The most common clinical presentations were renal failure,abdominal pain,and body weight loss.There were no significant differences of Hb,Scr,ESR and CRP between the IgG4-RPF and non-IgG4-RPF patients.CT or MRI scans showed retroperitoneal masses in all 16 cases,however,muti-organs injuries,such as submandibular gland,deep lymph nodes,pancreas,bile ducts,thymus glands,renal interstitium and thyroid glands,were common in IgG4-RPF patients.Pathological investigations displayed that all patients presented the same alterations,such as the elevated IgG4 ＋ plasm cells,IgG4 ＋/IgG ratio of cells and storiform fibrosis,in vary involved organs.8 patients were treated with corticosteroids and immunosuppressive agents;6 patients were treated with corticosteroids and surgical intervention.These 14 patients presented good response to corticosteroids,except 2 cases who relapsed after corticosteroids withdrawal.2 patients who accpted surgical intervention,and there was the risk of repeated episodes.Conclusions IgG4-related disease accounts for a substantial percentage of patients with idiopathic RPF.Histopathologic features such as elevated IgG4/total IgG ratios,IgG4 ＋ plasma cells per high-power field and storiform fibrosis are critical to identify this disease.