Guillevin Loïc, Terrier Benjamin
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, INSERM U1060, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, University of Paris 5-René-Descartes, Paris, France.
Int J Rheum Dis. 2019 Jan;22 Suppl 1:100-104. doi: 10.1111/1756-185X.13303. Epub 2018 Apr 19.
The classification of the systemic vasculitides has been controversial for several decades. The Chapel Hill consensus Conference definitions originally developed in 1994, but revised and extended in 2012 are now widely accepted. The American College of Rheumatology (ACR) criteria were first published in 1990, are now generally accepted to be out of date and new criteria are needed. More recently the classical division of the ANCA vasculitides using clinical phenotype has come under scrutiny with evidence from epidemiological, genetic and outcome studies that perhaps these conditions should be classified on the basis of ANCA specificity into PR3-ANCA positive and MPO-ANCA positive groups. The traditional distinction between giant cell arteritis and Takayasu arteritis has been questioned and some recent studies of GCA have included patients with only extra-cranial disease. The Diagnostic and Classification Criteria of Vasculitis study (DCVAS) will provide new validated classification criteria for the systemic vasculitides.
几十年来，系统性血管炎的分类一直存在争议。1994年最初制定、2012年修订并扩展的《 Chapel Hill共识会议定义》目前已被广泛接受。美国风湿病学会（ACR）标准于1990年首次发布，现在普遍认为已过时，需要新的标准。最近，使用临床表型对ANCA血管炎进行的经典分类受到了审视，流行病学、遗传学和结局研究的证据表明，也许这些疾病应根据ANCA特异性分为PR3-ANCA阳性组和MPO-ANCA阳性组。巨细胞动脉炎和大动脉炎之间的传统区别受到质疑，最近一些关于巨细胞动脉炎的研究纳入了仅患有颅外疾病的患者。血管炎诊断和分类标准研究（DCVAS）将为系统性血管炎提供新的经过验证的分类标准。
