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.
过敏性紫癜(Henoch-Schönlein purpura, HSP)又称为IgA血管炎,是由IgA免疫复合物沉积介导的全身性小血管炎。HSP以儿童最常见,成人较为少见。临床上以皮肤紫癜最多见,可伴有关节、胃肠道及肾脏等各系统症状。当腹部症状先于紫癜出现时,易被缺乏相关经验的临床医生误诊为急腹症、急性阑尾炎、消化道出血等疾病。现报道1例以腹部症状为主的成人混合型HSP,目的在于提高临床医生对HSP的诊治水平,避免延误患者的诊治。Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, is a systemic small vasculitis mediated by IgA immune complex deposition. HSP is most common in children and less common in adults. The most common clinical skin purpura, can be accompanied by joint, gastrointestinal and kidney and other system symptoms. When the abdominal symptoms appear before purpura, it is easy to be misdiagnosed as acute abdomen, acute appendicitis, gastrointestinal bleeding and other diseases by clinicians who lack relevant experience. A case of adult mixed HSP with abdominal symptoms was reported in order to improve the diagnosis and treatment of HSP by clinicians and avoid delay in diagnosis and treatment.
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