系统性红斑狼疮(SLE)是一种影响多个器官的全身性自身免疫疾病,其特点是免疫细胞、免疫因子和免疫途径的复杂相互作用导致各种临床表现。抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)是一组以中小血管坏死性炎症和外周血中出现ANCA为特征的自身免疫综合征。SLE/AAV重叠综合征(overlap syndrome, OS)一种混合表现的炎症性疾病,具有SLE和AAV的临床特征。在极少数情况下,SLE和AAV同时发病表现出更严重的临床症状。缺血性结肠炎(IC)是一组存在血管闭塞性或非闭塞性疾病的临床综合征,以结肠供血不足为特征。本文报告了1例SLE/AAV OS伴随缺血性结肠炎患者。经过血浆置换、足量激素冲击、免疫抑制剂环磷酰胺和硫酸羟氯喹、抑酸药、生长激素抑制类药物等治疗后,患者病情缓解。Systemic lupus erythematosus (SLE) is a systemic autoimmune disease that affects multiple organs and is characterized by a complex interplay of immune cells, immune factors, and immune pathways leading to a variety of clinical manifestations. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune syndromes characterized by necrotizing inflammation of small and medium-sized blood vessels and the presence of ANCA in the peripheral blood. Overlap syndrome (OS) is a mixed manifestation of inflammatory disease with clinical features of SLE and AAV. In rare cases, the concurrent onset of SLE and AAV results in SLE/AAV OS, which exhibits more severe clinical symptoms. Ischemic colitis (IC) is a group of clinical syndromes in the presence of vaso-occlusive or non-occlusive disorders characterized by inadequate blood supply to the colon. In this article, we report a patient with SLE/AAV OS combined with ischemic colitis. After treatment with adequate hormonal shocks, the immunosuppressants cyclophosphamide and hydroxychloroquine sulfate, plasma exchange, acid-suppressing drugs, and anti-growth hormone analogs,
1病例资料患者男性,59岁。因"背部疼痛7小时"入院。患者7小时前无明显诱因出现背部剧烈抽搐样疼痛,逐渐加重,休息后未缓解,伴大汗淋漓,遂前往我院就诊。既往有高血压病、脑梗塞、慢性肾脏病史。吸烟史10余年,5~6支/天。查体:心率79次/分,血压200/120 mm Hg,余未见明显异常。心电图大致正常,心脏彩超示:左房增大,左室舒张功能降低。LVEF:62%;E/A<1。主动脉增强CT示主动脉夹层(DeBakeyⅢ型),假腔内少量血栓形成,主动脉、双髂动脉粥样硬化并多发溃疡形成。双肾动脉粥样硬化,管腔重度狭窄。
