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...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, the patient's condition resolved.展开更多
目的探讨抗中性粒细胞胞浆抗体(ANCA)相关血管炎性中耳炎(otitis media with antineutrophil cytoplasmic antibody-associated vasculitis,OMAAV)的临床特点及诊治方法。方法在中国知网、维普、万方等中文数据库中,检索以分泌性中耳炎...目的探讨抗中性粒细胞胞浆抗体(ANCA)相关血管炎性中耳炎(otitis media with antineutrophil cytoplasmic antibody-associated vasculitis,OMAAV)的临床特点及诊治方法。方法在中国知网、维普、万方等中文数据库中,检索以分泌性中耳炎为首发症状的ANCA相关血管炎临床病例,结合1例经治的OMAAV病例,总结其临床特点、诊治及预后。结果检索到以分泌性中耳炎为首发症状的ANCA相关血管炎相关文献5篇病例7例,加上本研究的1例(简称“本例”)共8例患者,均以分泌性中耳炎为首发症状,其中3例继发耳痛,3例继发鼓膜紧张部穿孔,1例出现面瘫。除本例患者外,其余7例均在出现发热、肺部结节等耳部以外表现后方得以确诊,确诊时间1月~3年,平均10.3月,其中5例确诊依据是耳部以外组织活检加ANCA血清学检查,2例是临床表现加ANCA血清学检查;2例死于ANCA相关血管炎,其余5例经治疗后病情缓解。本例患者根据日本耳科学会最近提出的OMAAV诊断标准确诊并及时给予激素和硫唑嘌呤治疗,预后良好。结论以分泌性中耳炎、周围性面瘫及耳痛等耳部症状为首发症状的ANCA相关血管炎早期临床症状不典型,极易误诊。建议根据OMAAV相关诊断标准早期进行规范诊治,减少误诊漏诊,改善预后。展开更多
文摘系统性红斑狼疮(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, the patient's condition resolved.
文摘目的探讨抗中性粒细胞胞浆抗体(ANCA)相关血管炎性中耳炎(otitis media with antineutrophil cytoplasmic antibody-associated vasculitis,OMAAV)的临床特点及诊治方法。方法在中国知网、维普、万方等中文数据库中,检索以分泌性中耳炎为首发症状的ANCA相关血管炎临床病例,结合1例经治的OMAAV病例,总结其临床特点、诊治及预后。结果检索到以分泌性中耳炎为首发症状的ANCA相关血管炎相关文献5篇病例7例,加上本研究的1例(简称“本例”)共8例患者,均以分泌性中耳炎为首发症状,其中3例继发耳痛,3例继发鼓膜紧张部穿孔,1例出现面瘫。除本例患者外,其余7例均在出现发热、肺部结节等耳部以外表现后方得以确诊,确诊时间1月~3年,平均10.3月,其中5例确诊依据是耳部以外组织活检加ANCA血清学检查,2例是临床表现加ANCA血清学检查;2例死于ANCA相关血管炎,其余5例经治疗后病情缓解。本例患者根据日本耳科学会最近提出的OMAAV诊断标准确诊并及时给予激素和硫唑嘌呤治疗,预后良好。结论以分泌性中耳炎、周围性面瘫及耳痛等耳部症状为首发症状的ANCA相关血管炎早期临床症状不典型,极易误诊。建议根据OMAAV相关诊断标准早期进行规范诊治,减少误诊漏诊,改善预后。
