Ernst Diana, Bearlecken Niklas, Ernst Schmidt Reinhold, Witte Torsten
Department of Rheumatology & Immunology, Hannover Medical University, Hannover, Germany.
Open Rheumatol J. 2016 Nov 30;10:101-108. doi: 10.2174/1874312901610010101. eCollection 2016.
Whilst large vessel vasculitis (LVV) predominantly occurs in isolation, associations with other infectious and non-infectious diseases have been reported. Limited data describing associations with various autoimmune diseases (AI), including spondyloarthritis exists. The aim of this study was to characterize the association of LVV and spondyloarthritis or its associated diseases (SpAD).
A single centre, retrospective study of patients ≥50yrs with first presentation LVV between 01.06.2008-01.06.2015 was performed. Patients were categorized according to SpA or associated disease, other AI or idiopathic LVV (iLVV). Clinical, laboratory and imaging findings were compared. Kaplan-Meyer survival analysis, with relapse taken as the primary end-point, was performed.
LVV was confirmed in 62 pts, of who 16/62 (26%) had SpA or associated disease. In these patients, LVV presented earlier (59.2 SpAD . 68.1 AI and 70.3yrs iLVV; p=0.01) and occurred predominantly in spring compared to autumn and winter in non-SpA patients, was associated with more centralised pattern of distribution (p=0.05) and was more likely to exhibit a refractory course (p=0.05). Overall relapse rates were similar across groups. Smoking-status influenced age of onset in all groups, being associated with earlier onset.
A clear association between LVV and SpAD exists. LVV associated with SpAD has a particular phenotype characterised by earlier onset, thorax-limited disease and increased risk of a refractory course. Given on-going LVV treatment trials further genetic and pathophysiological characterization appears warranted, to evaluate potential variation in treatment response and optimize future care.
虽然大血管血管炎（LVV）主要单独发生，但也有与其他感染性和非感染性疾病相关的报道。关于与包括脊柱关节炎在内的各种自身免疫性疾病（AI）相关的描述数据有限。本研究的目的是描述LVV与脊柱关节炎或其相关疾病（SpAD）之间的关联。
对2008年6月1日至2015年6月1日首次出现LVV的≥50岁患者进行单中心回顾性研究。患者根据脊柱关节炎或相关疾病、其他AI或特发性LVV（iLVV）进行分类。比较临床、实验室和影像学检查结果。以复发为主要终点进行Kaplan-Meier生存分析。
62例患者确诊为LVV，其中16/62（26%）患有脊柱关节炎或相关疾病。在这些患者中，LVV出现较早（SpAD患者59.2岁，AI患者68.1岁，iLVV患者70.3岁；p=0.01），与非脊柱关节炎患者相比，主要在春季发病，与更集中的分布模式相关（p=0.05），并且更有可能表现为难治性病程（p=0.05）。各组的总体复发率相似。吸烟状况影响所有组的发病年龄，与发病较早相关。
LVV与SpAD之间存在明确关联。与SpAD相关的LVV具有特定的表型，其特征为发病较早、胸部局限性疾病以及难治性病程风险增加。鉴于正在进行的LVV治疗试验，进一步的基因和病理生理特征分析似乎是必要的，以评估治疗反应的潜在差异并优化未来的治疗。
