重叠型系统性红斑狼疮、血清阳性侵蚀性类风湿性关节炎和ANCA相关血管炎的病例报告和文献综述。

IF 0.9 Q4 RHEUMATOLOGY Modern rheumatology case reports Pub Date : 2023-12-29 DOI:10.1093/mrcr/rxad056
Emma Reesor, Sankalp Virendrakumar Bhavsar
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引用次数: 0

摘要

类风湿性关节炎和系统性红斑狼疮的重叠有很好的描述,其综合征被称为“rhupus”。然而,ANCA相关血管炎与其他自身免疫性疾病罕见相关。在这里,我们报告了一例狼疮、类风湿性关节炎和ANCA相关血管炎,两次发作之间相隔很长时间,导致这三种诊断在35年的过程中累积。患者在45岁时被诊断为狼疮,表现为炎症性多关节炎、血小板减少、白细胞减少、ANA阳性、抗DNA阳性和低补体血症。她继续接受羟氯喹治疗,疾病活动性很小。大约20年后,她突发多关节炎,伴有双侧手腕侵蚀性关节病和类风湿因子阳性,被诊断为类风湿性关节炎。开始了抗TNFα治疗,她在接下来的10年里病情稳定。79岁时,她患上了ANCA相关血管炎AAV,伴有肺部和肾脏表现,接受了利妥昔单抗诱导治疗和类固醇治疗。她康复了,MPO-ANCA滴度正常。一年后,在停用抗TNFα后,她再次经历了急性肾损伤,MPO-ANCA重复升高。她被再次诱导,并继续服用类固醇和利妥昔单抗。这一新病例强调了可能的重叠综合征的范围,以及多种自身免疫诊断在过去几十年中如何在以前稳定的疾病中演变。围绕ANCA相关血管炎和其他系统性自身免疫性疾病的多自身免疫性研究越来越多,这有可能确定常见的病因和发病机制。了解这些重叠综合征有助于及时识别和管理这些情况。
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Overlapping systemic lupus erythematosus, seropositive erosive rheumatoid arthritis, and ANCA-associated vasculitis: A case report and literature review.

The overlap of rheumatoid arthritis and systemic lupus erythematosus is well described, with a syndrome known as 'rhupus'. ANCA-associated vasculitis, however, is uncommonly associated with other autoimmune conditions. Here, we present a case of lupus, rheumatoid arthritis, and ANCA-associated vasculitis, with significant time elapsed between presentations, resulting in an accumulation of the three diagnoses over the course of 35 years. The patient was diagnosed with lupus at age 45, presenting with inflammatory polyarthritis, thrombocytopenia, leukopenia, positive ANA, positive anti-DNA, and hypocomplementemia. She was maintained on hydroxychloroquine therapy with minimal disease activity. Approximately 20 years later, she had a flare of polyarthritis with bilateral wrist erosive arthropathy and a positive rheumatoid factor, diagnosed as rheumatoid arthritis. Anti-TNFα therapy was initiated, and she was stable for a further 10 years. At age 79, she developed ANCA-associated vasculitis AAV with pulmonary and renal manifestations, treated with rituximab induction therapy and steroids. She recovered and her MPO-ANCA titre normalised. One year later, off the anti-TNFα, she again experienced an acute kidney injury with a repeat rise in MPO-ANCA. She was re-induced and maintained on steroids and rituximab. This novel case highlights the range of possible overlap syndromes, as well as how multiple autoimmune diagnoses can evolve over decades in previously stable disease. There is growing work around polyautoimmunity with ANCA-associated vasculitis and other systemic autoimmune diseases, which has potential to identify common aetiologies and pathogenesis. Knowledge of these overlap syndromes can help to recognise and manage these conditions in a timely manner.

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