慢性主动脉周围炎临床分析

Analysis of clinical features of chronic periaortitis

目的了解我国慢性主动脉周围炎患者的临床特点,提高对该疾病的认识。方法对28例确诊慢性主动脉周围炎患者的临床资料进行回顾性分析,28例中男∶女=4·5∶1,平均年龄52·8岁。结果28例中20例(71·4%)为特发性腹膜后纤维化,5例(17·8%)为炎性腹主动脉瘤,3例(10·7%)为主动脉瘤周围腹膜后纤维化。最常见的症状是腹痛9例(35·7%)、腰痛7例(25%)、体重减轻7例(25%)、腹胀5例(17·8%)和下肢浮肿5例(17·8%)。CT和MRI检查发现腹膜后软组织影。10例患者是经病理学检查确诊。17例(85%)特发性腹膜后纤维化患者经B超声检查发现有肾积水,7例经B超声检查发现为腹主动脉扩张。20例中有8例进行手术治疗,17例采用糖皮质激素和/或免疫抑制剂和/或他莫昔芬治疗。1例合并强直性脊柱炎,1例合并未分化结缔组织疾病。无并发其他器官纤维化疾病者。5例炎性腹主动脉瘤患者均有典型影像学表现,其中3例经病理学检查证实。3例动脉瘤周围腹膜后纤维化患者均被误诊为特发性腹膜后纤维化或腹主动脉瘤。结论慢性主动脉周围炎是一种罕见自身免疫性风湿病,以特发性腹膜后纤维化为最多见。无特异临床症状,但可以合并自身免疫性风湿病或自身抗体,确诊有赖于影象学证据和病理学检查。糖皮质激素联合免疫抑制剂或他莫昔芬可以缓解病情。

Objective Understand the clinical features of chronic periaortitis. Methods The medical records of 28 cases with definite diagnosis of chronic periaortitis were reviewed retrospectively. Results Among these 28 cases, 20（71.4% ） fulfilled the diagnostic criteria of idiopathic retroperitoneal fibrosis（IPF）, 5 （ 17. 8% ） were inflammatory abdominal aortic aneurysm （IAAA） and 3 （ 10. 7% ） were perianeurysmal retroperitoneal fibrosis （PARF）. The common symptoms were abdominal blunt pain （35.7%）, lumbago （25%）, loss of body weight （25%）, abdominal distension （ 17. 8% ） and lower extremities pitting edema（ 17. 8% ）. Hydronephrosis was found by B untrasonography in 17（85% ） patients with IPF. Dilated abdominal aorta were found in 7 patients. One case was complicated with ankylosing spondylitis and another one was diagnosed to have undifferentiated connective diseases. All 5 IAAA patients were treated by aneurysm segregation or stent implantation, but none had medical therapy. The 3 perianeurysmal retroperitoneal fibrosis patients were mis-diagnosed as with either IPF or AAA. Seventeen patients in this group were treated with corticosteroid combined with immunosuppressive agents or tamoxiferL Four cases were followed up and their conditions were improved which were demonstrated by repeated CT or MRI. Conclusion Chronic periaortitis is a rare autoimmune rheumatic disease. IPF is the most common one compared to IAAA and PARF. It can be complicated with autoimmune disorders or serum autoantibodies. No case with other organ fibrosis disorder wis observed in this study. Radiological examination and pathological examination are necessary for confirming the diagnosis. Corticosteroid combined with immunosuppressive agents or tamoxifen is the effective treatment.