Department of Internal Medicine - Multi-Organic Diseases, Local Referral Center for Rare Auto-immune Diseases, Montpellier University Hospital, Montpellier, France.
Department of nephrology, Université de Montpellier, CHU de Montpellier, Montpellier, France.
Autoimmun Rev. 2022 Jun;21(6):103099. doi: 10.1016/j.autrev.2022.103099. Epub 2022 Apr 19.
Sjögren's syndrome (SS) and ANCA-associated vasculitis (AAV) have distinct clinical presentation and evolution, with paucity of reports on overlap syndrome. We aimed to better characterize this entity.
We report four additional cases from the Montpellier university hospital. We also performed a systematic literature review, according to PRISMA guidelines, in Medline, Embase, Web of science, Cochrane Library, and grey literature. Demographic, clinical, and paraclinical data on SS and AAV were analysed.
A total of 3133 articles was identified in databases, with 2695 articles screened for eligibility. After exclusion, we had 30 articles on 40 patients to analyse, in addition to 4 patients from our local recruitment (44 patients overall). Patients were female in 81.8%, with median age at AAV onset of 63.5 years. All patients but one presented with SS before, or concomitantly to the diagnosis of AAV, with a median delay of 12 months between both diagnoses. AAV predominantly had renal involvement (35/44 patients, 79.5%), anti-MPO antibodies being the most frequent (35 patients), even in patients presenting with granulomatosis with polyangiitis. We observed significantly more Raynaud phenomenon and associated auto-immune diseases in the group of non-granulomatous AAV (10 patients versus 1, p = 0.015 and 8 patients versus 0, p = 0.013, respectively).
This is the largest descriptive study on the association between SS and AAV, providing information on this challenging diagnosis and interplay between these two diseases. Particular attention should be paid in the first months after diagnosis, given the specific complications and outcomes of each disease.
干燥综合征（SS）和抗中性粒细胞胞浆抗体相关性血管炎（AAV）的临床表现和演变明显不同，重叠综合征的报道较少。我们旨在更好地描述这种疾病。
我们报告了来自蒙彼利埃大学医院的另外 4 例病例。我们还按照 PRISMA 指南，在 Medline、Embase、Web of science、Cochrane Library 和灰色文献中进行了系统的文献回顾。分析了 SS 和 AAV 的人口统计学、临床和实验室数据。
在数据库中总共确定了 3133 篇文章，对 2695 篇文章进行了筛选以确定其是否符合入选标准。排除后，我们共分析了 30 篇文章中的 40 例患者，此外还有 4 例来自我们当地的招募（总共 44 例患者）。患者中女性占 81.8%，AAV 发病时的中位年龄为 63.5 岁。除了 1 例患者外，所有患者均在 AAV 诊断前或同时出现 SS，两种诊断之间的中位时间间隔为 12 个月。AAV 主要累及肾脏（44 例患者中的 35 例），最常见的是抗髓过氧化物酶抗体（35 例患者），甚至在出现肉芽肿性多血管炎的患者中也是如此。我们观察到，在非肉芽肿性 AAV 组中，雷诺现象和相关自身免疫性疾病明显更多（10 例与 1 例，p=0.015 和 8 例与 0 例，p=0.013）。
这是关于 SS 和 AAV 之间关联的最大描述性研究，提供了有关这种具有挑战性的诊断和两种疾病之间相互作用的信息。鉴于每种疾病的特定并发症和结局，应在诊断后的最初几个月特别注意。
