From Sorbonne Universités, UPMC Univ Paris 06, Inflammation-Immunopathology-Biotherapy Department, France (C.C., P.C, D.S.); INSERM, Paris, France (C.C., P.C, D.S.); Centre National de la Recherche Scientifique, Paris, France (C.C., P.C, D.S.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires, Paris, France (C.C., Y.F., P.C, D.S.); AP-HP, SBIM, Hôpital Saint-Louis, Université Paris Diderot, France (L.B., M.R.-R.); INSERM, ECSTRA Team, Paris, France (L.B., M.R.-R.); Hôpital Claude Huriez, Service de Médecine Interne, Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, CHRU, Université de Lille, France (M.L., E.H.); AP-HP, Service de Médecine Interne, Hôpital Saint-Antoine, UPMC, Paris France (A.M.); Service de Médecine Interne, Hôpital Foch, Suresnes, France (J.-E.K.); Service de Médecine Interne, CHU de Rouen, France (Y.B.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Chirurgie Vasculaire, UPMC, Paris, France (L.C., F.K.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Département d'Imagerie CardioVasculaire et de Radiologie Interventionnelle, UPMC, INSERM-Centre National de la Recherche Scientifique-LIB, Paris, France (P.C.); AP-HP, Hôpital Européen Georges-Pompidou, Service de Médecine Vasculaire, INSERM UMR970, PARCC, Centre de Référence des Maladies Vasculaires Rares, DHU Pathologies Artérielles Rares et Communes, Hôpitaux Universitaires Paris Ouest, Université Paris Descartes, Sorbonne Paris Cité, France (E.M., T.M.).
Circulation. 2017 Sep 19;136(12):1114-1122. doi: 10.1161/CIRCULATIONAHA.116.027094. Epub 2017 Jul 12.
Because of the wide variation in the course of Takayasu arteritis (TA), predicting outcome is challenging. We assess long-term outcome and prognosis factors for vascular complications in patients with TA.
A retrospective multicenter study of characteristics and outcomes of 318 patients with TA fulfilling American College of Rheumatology and Ishikawa criteria was analyzed. Factors associated with event-free survival, relapse-free survival, and incidences of vascular complications were assessed. Risk factors for vascular complications were identified in a multivariable model.
The median age at TA diagnosis was 36 [25-47] years, and 276 patients (86.8%) were women. After a median follow-up of 6.1 years, relapses were observed in 43%, vascular complications in 38%, and death in 5%. Progressive clinical course was observed in 45%, carotidodynia in 10%, and retinopathy in 4%. The 5- and 10-year event-free survival, relapse-free survival, and complication-free survival were 48.2% (42.2; 54.9) and 36.4% (30.3; 43.9), 58.6% (52.7; 65.1) and 47.7% (41.2; 55.1), and 69.9% (64.3; 76.0) and 53.7% (46.8; 61.7), respectively. Progressive disease course (=0.018) and carotidynia (=0.036) were independently associated with event-free survival. Male sex (=0.048), elevated C-reactive protein (=0.013), and carotidynia (=0.003) were associated with relapse-free survival. Progressive disease course (=0.017), thoracic aorta involvement (=0.009), and retinopathy (=0.002) were associated with complication-free survival.
This nationwide study shows that 50% of patients with TA will relapse and experience a vascular complication ≤10 years from diagnosis. We identified specific characteristics that identified those at highest risk for subsequent vascular complications.
由于 Takayasu 动脉炎（TA）的病程差异很大，因此预测其预后具有挑战性。我们评估了 TA 患者血管并发症的长期结局和预后因素。
对符合美国风湿病学会和石川标准的 318 例 TA 患者的特征和结局进行回顾性多中心研究。评估了与无事件生存、无复发生存和血管并发症发生率相关的因素。在多变量模型中确定了血管并发症的危险因素。
TA 诊断时的中位年龄为 36 [25-47] 岁，276 例（86.8%）为女性。中位随访 6.1 年后，43%的患者出现复发，38%的患者出现血管并发症，5%的患者死亡。45%的患者出现进展性临床病程，10%的患者出现颈动脉痛，4%的患者出现视网膜病变。5 年和 10 年无事件生存率、无复发生存率和无并发症生存率分别为 48.2%（42.2；54.9）和 36.4%（30.3；43.9），58.6%（52.7；65.1）和 47.7%（41.2；55.1），69.9%（64.3；76.0）和 53.7%（46.8；61.7）。进展性疾病过程（=0.018）和颈动脉痛（=0.036）与无事件生存独立相关。男性（=0.048）、C 反应蛋白升高（=0.013）和颈动脉痛（=0.003）与无复发生存相关。进展性疾病过程（=0.017）、胸主动脉受累（=0.009）和视网膜病变（=0.002）与无并发症生存相关。
这项全国性研究表明，50%的 TA 患者在诊断后 10 年内会复发并发生血管并发症。我们确定了一些特定的特征，可以识别出那些发生后续血管并发症风险最高的患者。
