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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 To understand the clinical features of patients with chronic aorta inflammation in our country and to improve their understanding of the disease. Methods The clinical data of 28 patients diagnosed as chronic aortic periarthritis were retrospectively analyzed. Among them, 28 males: 4 females = 4. 5: 1, with an average age of 52.8 years. Results Twenty of the 28 patients (71.4%) had idiopathic retroperitoneal fibrosis, 5 (17.8%) had inflammatory abdominal aortic aneurysm and 3 (10.7%) had aortic aneurysm Retroperitoneal fibrosis. The most common symptoms were abdominal pain (7 · 7%), low back pain (25%), weight loss (7%), abdominal distension (17.8%) and lower extremity edema %). CT and MRI examination of retroperitoneal soft tissue findings. Ten patients were confirmed by pathology. Seventeen (85%) patients with idiopathic retroperitoneal fibrosis had hydronephrosis by B-ultrasound and seven had abdominal aorta dilatation by B-ultrasound. Eight of 20 patients underwent surgery and 17 were treated with glucocorticoids and / or immunosuppressive agents and / or tamoxifen. One patient had ankylosing spondylitis and one patient had undifferentiated connective tissue disease. No complicated with other organ fibrosis disease. Five patients with inflammatory abdominal aortic aneurysm had typical imaging findings, of which 3 were confirmed by pathological examination. 3 cases of patients with retroperitoneal fibrosis around the aneurysm were misdiagnosed as idiopathic retroperitoneal fibrosis or abdominal aortic aneurysm. Conclusion Chronic aortic peri-inflammation is a rare autoimmune rheumatism with idiopathic retroperitoneal fibrosis as the most common. No specific clinical symptoms, but can be combined with autoimmune rheumatoid disease or autoantibodies, diagnosis depends on the imaging evidence and pathological examination. Glucocorticoid combined with immunosuppressive agents or tamoxifen can ease the disease.