在引入有效的治疗之前，未经治疗的系统性肉芽肿性多血管炎 (GPA)（以前称为韦格纳肉芽肿）患者的中位生存时间为 5 个月，大部分在 12 月内死亡。目前，有了积极的免疫抑制方案，大多数患者可以获得缓解。然而，尽管有这些进步，但疾病以及治疗相关的并发症和死亡仍然是重要问题。[36]Little MA, Nightingale P, Verburgh CA, et al; European Vasculitis Study (EUVAS) Group. Early mortality in systemic vasculitis: relative contribution of adverse events and active vasculitis. Ann Rheum Dis. 2010 Jun;69(6):1036-43.http://www.ncbi.nlm.nih.gov/pubmed/19574233?tool=bestpractice.com[37]Mohammad AJ, Jacobsson LT, Westman KW, et al. Incidence and survival rates in Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford). 2009 Dec;48(12):1560-5.http://www.ncbi.nlm.nih.gov/pubmed/19797309?tool=bestpractice.com 所有患者中大约有一半人将经历至少一次疾病复发。[38]Walsh M, Faurschou M, Berden A, et al; European Vasculitis Study Group. Long-term follow-up of cyclophosphamide compared with azathioprine for initial maintenance therapy in ANCA-associated vasculitis. Clin J Am Soc Nephrol. 2014 Sep 5;9(9):1571-6.http://www.ncbi.nlm.nih.gov/pubmed/24970876?tool=bestpractice.com 此外，绝大多数患者将发生至少一种与 GPA 和/或其治疗相关的并发症。[39]Seo P, Min YI, Holbrook JT, et al; WGET Research Group. Damage caused by Wegener's granulomatosis and its treatment: prospective data from the Wegener's Granulomatosis Etanercept Trial. Arthritis Rheum. 2005 Jul;52(7):2168-78.https://onlinelibrary.wiley.com/doi/full/10.1002/art.21117http://www.ncbi.nlm.nih.gov/pubmed/15986348?tool=bestpractice.com 一项大型多中心队列研究的数据表明，死亡的主要原因是感染、心血管疾病和活动性血管炎。[40]Flossmann O, Berden A, de Groot K, et al; European Vasculitis Study Group. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011 Mar;70(3):488-94.http://www.ncbi.nlm.nih.gov/pubmed/21109517?tool=bestpractice.com
