Department of Medicine, Division of Rheumatology, McMaster University, Hamilton, ON, Canada.
Internal Medicine, McMaster University, Waterloo, ON, Canada.
Mod Rheumatol Case Rep. 2023 Dec 29;8(1):21-25. doi: 10.1093/mrcr/rxad056.
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.
类风湿关节炎和系统性红斑狼疮的重叠表现已有充分描述，有一种综合征称为“rhupus”。然而，抗中性粒细胞胞质抗体（ANCA）相关性血管炎很少与其他自身免疫性疾病相关。在此，我们报告了一例狼疮、类风湿关节炎和 ANCA 相关性血管炎的病例，这些疾病的表现时间间隔较长，导致这三种诊断在 35 年的时间里逐渐积累。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、抗核抗体阳性、抗 DNA 抗体阳性和补体水平降低。她接受羟氯喹治疗，疾病活动度较低。大约 20 年后，她出现多关节炎发作，双侧腕关节侵蚀性关节炎，类风湿因子阳性，被诊断为类风湿关节炎。她开始接受抗 TNF-α 治疗，病情稳定了 10 年。79 岁时，她出现了伴有肺和肾表现的 ANCA 相关性血管炎，接受了利妥昔单抗诱导治疗和类固醇治疗。她康复了，MPO-ANCA 滴度恢复正常。一年后，停用抗 TNF-α 后，她再次出现急性肾损伤，MPO-ANCA 再次升高。她再次接受诱导治疗，并维持使用类固醇和利妥昔单抗。这个新病例突出了可能的重叠综合征的范围，以及在先前稳定的疾病中，多个自身免疫性诊断如何在几十年中逐渐演变。目前，围绕着 ANCA 相关性血管炎和其他系统性自身免疫性疾病的多自身免疫性，已经有越来越多的研究，这有可能确定共同的病因和发病机制。了解这些重叠综合征有助于及时识别和管理这些疾病。
