特发性腹膜后纤维化七例临床分析

目的：加深对特发性腹膜后纤维化临床体征的认识,提高早期诊断的水平。方法：总结我院近十年来7例特发性腹膜后纤维化病人的临床情况,实验室检查及CT,核磁共振检查的特点,结果：（1）临床表现：7例病人的首发症状各异,但以腰背痛,腹痛及消瘦为多见,后期症状因受累脏器的不同而异,其中以腹部包块,腹水,泌尿系统症状及肠梗阻常见。（2）诊断：根据病人血红细胞沉降率增快,γ球蛋白升高及CT和核磁共振阳性表现为诊断依据。腹部CT检查对于腹部肿块和脏器粘连比B超检查敏感。但合并肾积水及输尿管病变的病人则以B超检查最为优势。（3）治疗：治疗的效果与疾病发展的程度,阶段有关,当疾病在炎症活动期,应积极给予足量激素和免疫抑制剂治疗;当纤维化已形成并发展到梗阻时,需及时手术治疗。结论：影像学检查对特发性腹膜后纤维化的诊断非常重要。但最后确诊需手术和病理学检查。

Idiopathic retroperitoneal fibrosis: a clinical analysis of 7 cases

Objective To investigate the clinical characteristics of idiopathic retroperitoneal fibrosis. Methods The clinical manifestations, laboratory examination, and CT and MIR features of seven patients with idiopathic retroperitoneal fibrosis were analyzed. Results (1) The initial symptoms were nonspecific and variable and included abdominal pain, low back pain, and weigh loss. Later symptoms, depending on the organs affected, included abdominal mass, ascites, urinary symptoms, and and intestinal obstruction. (2) Diagnosis of IRF could be based on elevated erythrocyte sedimentatio rate and gamma globulin and positive findings by CT and MIR. For masses in retroperitoneal space and adhesion of abdominal organs, CT was more sensitive than B mode untrasonography. However, for hydronephrosis and lesions in ureter, B mode ultrasonography was the first choice. (3) Steroid and immunosupressant were effective during the early stage of IRF. In the advanced stage, operation was necessary. Conclusion Imaging examination is important for diagnosis of IRF. However, the final diagnosis depends on patjological examination. Treatment depends on the stage of disease. The prognosis is optimistic.