目的:干燥综合征(SS)和ANCA相关性血管炎(AAV)具有不同的临床表现和演变,缺乏重叠综合征的报道。我们旨在更好地描述这个实体。
方法:我们报告了蒙彼利埃大学医院另外4例病例。我们还进行了系统的文献综述,根据PRISMA指南,在Medline,Embase,WebofScience,科克伦图书馆,灰色文学人口统计,临床,并对SS和AAV的临床数据进行了分析。
结果:数据库中总共发现了3133篇文章,2695篇文章被筛选为合格。排除后,我们有30篇关于40个病人的文章要分析,除了我们当地招募的4名患者(总共44名患者)。81.8%的患者为女性,AAV发病的中位年龄为63.5岁。除一名患者之前出现SS外,所有患者,或伴随着AAV的诊断,两次诊断之间的中位延迟为12个月。AAV主要有肾脏受累(35/44例,79.5%),抗MPO抗体是最常见的(35名患者),即使在出现肉芽肿性多血管炎的患者中。我们在非肉芽肿性AAV组中观察到明显更多的雷诺现象和相关的自身免疫性疾病(分别为10例患者对1,p=0.015和8例患者对0,p=0.013)。
结论:这是关于SS和AAV之间关联的最大的描述性研究,提供有关这两种疾病之间具有挑战性的诊断和相互作用的信息。诊断后的头几个月应特别注意,考虑到每种疾病的具体并发症和结局。
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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).
CONCLUSIONS: 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.