Hočevar Alojzija, Rotar Ziga, Jurčić Vesna, Pižem Jože, Čučnik Saša, Vizjak Alenka, van den Broeke Rianne, Tomšič Matija
Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta 62, 1000, Ljubljana, Slovenia.
Institute of Pathology, Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1104, Ljubljana, Slovenia.
Arthritis Res Ther. 2016 Mar 2;18:58. doi: 10.1186/s13075-016-0959-4.
In 2010, EULAR/PRINTO/PRES proposed new classification criteria for paediatric IgA vasculitis (IgAV) that have a higher diagnostic sensitivity than the 1990 ACR criteria. These criteria have so far not been evaluated in adults, in whom IgAV is considered as a rare disease. Our main objective was to compare the diagnostic performance of EULAR/PRINTO/PRES and ACR classification criteria in adult IgAV.
Adult IgAV cases fulfilling the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (ICHCCNV) definition of IgAV at a secondary/tertiary rheumatology referral centre were critically reviewed in a partially retrospective and partially prospective manner. First, we compared the diagnostic sensitivity of ACR and EULAR/PRINTO/PRES criteria in this group of patients. Second, the diagnostic specificity of ACR and EULAR/PRINTO/PRES was determined by applying these criteria to a control group of patients with other systemic vasculitides.
Between 1 January 2010 and 31 December 2014 350 new cases of systemic vasculitis were identified. IgAV was diagnosed in 129, and other systemic vasculitides in 221 (123 had large, six medium and 92 small vessel vasculitis) cases according to ICHCCNV. The diagnostic sensitivity and specificity of the IgAV EULAR/PRINTO/PRES criteria were 99.2 % (95 % CI 95.4-99.9 %) and 86.0 % (95 % CI 80.7-90.3 %), and of the ACR criteria 86.8 % (95 % CI 79.7-92.1 %) and 81.0 % (95 % CI 75.2-85.9 %), respectively with an inter-criteria agreement of 77.5 % (95 % CI: 70.8-84.1 %).
In the adult population the EULAR/PRINTO/PRES IgAV classification criteria had a higher sensitivity and specificity than the ACR criteria.
2010年，欧洲抗风湿病联盟（EULAR）/国际儿童风湿病学和免疫学协会（PRINTO）/欧洲儿科风湿病学会（PRES）提出了小儿免疫球蛋白A血管炎（IgAV）的新分类标准，其诊断敏感性高于1990年美国风湿病学会（ACR）标准。迄今为止，这些标准尚未在成人中进行评估，成人IgAV被认为是一种罕见疾病。我们的主要目的是比较EULAR/PRINTO/PRES和ACR分类标准在成人IgAV中的诊断性能。
在一家二级/三级风湿病转诊中心，以部分回顾性和部分前瞻性的方式对符合2012年修订的国际教堂山共识会议血管炎命名法（ICHCCNV）中IgAV定义的成人IgAV病例进行了严格审查。首先，我们比较了ACR和EULAR/PRINTO/PRES标准在这组患者中的诊断敏感性。其次，通过将这些标准应用于其他系统性血管炎患者的对照组来确定ACR和EULAR/PRINTO/PRES的诊断特异性。
在2010年1月1日至2014年12月31日期间，共确定了350例新的系统性血管炎病例。根据ICHCCNV，129例诊断为IgAV，221例诊断为其他系统性血管炎（123例为大血管、6例为中血管和92例为小血管血管炎）。IgAV的EULAR/PRINTO/PRES标准的诊断敏感性和特异性分别为99.2%（95%置信区间95.4-99.9%）和86.0%（95%置信区间80.7-90.3%），ACR标准的诊断敏感性和特异性分别为86.8%（95%置信区间79.7-92.1%）和81.0%（95%置信区间75.2-85.9%），标准间一致性为77.5%（95%置信区间：70.8-84.1%）。
在成人中，EULAR/PRINTO/PRES的IgAV分类标准比ACR标准具有更高的敏感性和特异性。
