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,
目的:探讨血清骨唾液酸蛋白(BSP)与维持性血液透析(MHD)患者腹主动脉钙化的关系。方法:选取2016年5月至2017年2月于本院血液净化中心规律透析患者75例。检测患者血清BSP,并进行腹部侧位X平片拍摄,根据kauppila半定量积分方法进行腹主动脉钙化评分(AACs),参考CORD分段方法,根据AAC得分将患者分为无或轻度钙化组(AACs≤4)、中度钙化组(5≤AACs≤15)和重度钙化组(AACs≥16)。Logistic回归分析MHD患者AAC的影响因素。受试者工作特征(ROC)曲线分析BSP对血管钙化的诊断价值。结果:75例MHD患者中发生腹主动脉钙化者56例(74.67%),AACs中位数为4(0,24)分,血清BSP中位数为20.12(18.63,24.21)ng/ml。中度钙化组、重度钙化组BSP水平均高于无或轻度钙化组[22.43(19.58,6.84)ng/ml、21.99(19.87,26.18)ng/ml vs 19.16(17.3,23.3)ng/ml,P<0.05]。Logistic回归分析发现BSP是AAC的独立危险因素(OR=1.175,95%CI 1.004~1.375,P<0.05)。ROC曲线分析发现,BSP诊断AAC的曲线下面积为0.718(95%CI为0.604~0.833,P=0.001),BSP诊断AAC的临界值为21.51 ng/ml,灵敏度为0.711,特异度为0.595。结论:血清BSP水平和MHD患者AAC的严重程度相关,高水平的BSP是AAC的独立危险因素。BSP对MHD患者腹主动脉钙化具有潜在诊断价值。