疾病的缓解通常被定义为缺乏疾病的临床和实验室特征,且随访时进行的血管成像检查未发现新发血管病变的证据。[2]Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. 1995;21:1041-1058.http://www.ncbi.nlm.nih.gov/pubmed/8592736?tool=bestpractice.com[6]Maksimowicz-McKinnon K, Clark T, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000-1009.http://onlinelibrary.wiley.com/doi/10.1002/art.22404/fullhttp://www.ncbi.nlm.nih.gov/pubmed/17328078?tool=bestpractice.com虽然多数患者达到了疾病缓解,但大多数患者在糖皮质激素外还需联用免疫抑制疗法。[6]Maksimowicz-McKinnon K, Clark T, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000-1009.http://onlinelibrary.wiley.com/doi/10.1002/art.22404/fullhttp://www.ncbi.nlm.nih.gov/pubmed/17328078?tool=bestpractice.com约 20% 的患者的病程表现为单相。[2]Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. 1995;21:1041-1058.http://www.ncbi.nlm.nih.gov/pubmed/8592736?tool=bestpractice.com在一项研究中,当每日的泼尼松龙(泼尼松)剂量低于 10 mg 时,仅 28% 的患者得到大于 6 个月的持续缓解,而仅 17% 的患者在停用泼尼松龙(泼尼松)后仍持续缓解。[6]Maksimowicz-McKinnon K, Clark T, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000-1009.http://onlinelibrary.wiley.com/doi/10.1002/art.22404/fullhttp://www.ncbi.nlm.nih.gov/pubmed/17328078?tool=bestpractice.com
80% 以上达到疾病缓解的患者会出现疾病复发。[2]Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. 1995;21:1041-1058.http://www.ncbi.nlm.nih.gov/pubmed/8592736?tool=bestpractice.com[6]Maksimowicz-McKinnon K, Clark T, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000-1009.http://onlinelibrary.wiley.com/doi/10.1002/art.22404/fullhttp://www.ncbi.nlm.nih.gov/pubmed/17328078?tool=bestpractice.com即使持续使用免疫抑制治疗,也可能会出现复发。复发表现为血管成像检查中出现新发血管病变,通常伴有急性期标志物升高,但是可以缺乏这个活动性疾病的实验室证据。[6]Maksimowicz-McKinnon K, Clark T, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000-1009.http://onlinelibrary.wiley.com/doi/10.1002/art.22404/fullhttp://www.ncbi.nlm.nih.gov/pubmed/17328078?tool=bestpractice.com[40]Maksimowicz-McKinnon K, Hoffman GS. Takayasu arteritis: what is the long-term prognosis? Rheum Dis Clin North Am. 2007;33:777-786.http://www.ncbi.nlm.nih.gov/pubmed/18037116?tool=bestpractice.com
死亡率和发病率
心力衰竭是死亡的常见原因。[18]Mwipatayi BP, Jeffery PC, Beningfield SJ, et al. Takayasu arteritis: clinical features and management: report of 272 cases. ANZ J Surg. 2005;75:110-117.http://www.ncbi.nlm.nih.gov/pubmed/15777385?tool=bestpractice.com长期发病率主要与血管缺血引起的并发症有关。症状性肢体疼痛或不适可在约 50% 的患者中出现。与下肢相比,上肢疼痛或不适更为常见。胸主动脉瘤、主动脉瓣受累、冠状动脉炎和肺动脉炎是已知与死亡率增加相关的并发症。预计 Takayasu 动脉炎的 5 年死亡率在 70%-93% 之间。[41]Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S94-S104.http://www.ncbi.nlm.nih.gov/pubmed/19026150?tool=bestpractice.com
妊娠
由于大动脉炎主要在年轻女性中出现,所以经常要考虑到妊娠这个因素。虽然有关 Takayasu 动脉炎患者妊娠的数据很少,但已有成功妊娠的报道。[6]Maksimowicz-McKinnon K, Clark T, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000-1009.http://onlinelibrary.wiley.com/doi/10.1002/art.22404/fullhttp://www.ncbi.nlm.nih.gov/pubmed/17328078?tool=bestpractice.com[42]Vanoli M, Daina E, Salvarani C, et al. Takayasu's arteritis: a study of 104 Italian patients. Arthritis Rheum. 2005;53:100-107.http://onlinelibrary.wiley.com/doi/10.1002/art.20922/fullhttp://www.ncbi.nlm.nih.gov/pubmed/15696576?tool=bestpractice.com在一组患者中,确诊 Takayasu 动脉炎后,妊娠的年发生率下降,但流产的比例呈上升趋势。[42]Vanoli M, Daina E, Salvarani C, et al. Takayasu's arteritis: a study of 104 Italian patients. Arthritis Rheum. 2005;53:100-107.http://onlinelibrary.wiley.com/doi/10.1002/art.20922/fullhttp://www.ncbi.nlm.nih.gov/pubmed/15696576?tool=bestpractice.com妊娠期必须严密控制高血压。