Ishikawa K, Maetani S
Department of Internal Medicine, Faculty of Medicine, Kyoto University, Japan.
Circulation. 1994 Oct;90(4):1855-60. doi: 10.1161/01.cir.90.4.1855.
Patients with Takayasu's disease, a chronic inflammatory arteriopathy of unknown cause, have variable clinical courses, and predictors of the long-term outcome are not well understood. We studied prognostic factors of this disease, based on follow-up results, and a new prognostic classification was proposed.
Life-table methods and Cox regression analyses were applied to clinical data on 120 patients who had been prospectively followed for a median of 13 years (range, 1 month to 34 years). The overall survival rate at 15 years after the diagnosis was 82.9% and remained the same for the remainder of the follow-up period. Univariate Cox analyses revealed that of the six dichotomous variables evaluated at diagnosis, four were statistically significant predictors, including complications (Takayasu's retinopathy, hypertension, aortic regurgitation, and aneurysm), pattern of the past clinical course, age of the patient, and calendar year of diagnosis. Thus, the 15-year survival was 66.3% versus 96.4% for patients with and without a major complication, 67.9% versus 92.9% for patients with and without a progressive course, 58.3% versus 92.7% for age > 35 years and < or = 35 years, and 79.9% versus 96.5% for patients diagnosed in 1957 through 1975 and in 1976 through 1990, respectively. The delay in diagnosis and the erythrocyte sedimentation rate (ESR) were of marginal significance. The multivariate Cox analysis showed that only two of the above variables were statistically independent predictors, ie, the major complication and the progressive course. In addition to these two factors, ESR was the third predictor, if the Cox stepwise elimination procedure was performed. These three predictors used in various combinations made a total of 1822 classifications theoretically feasible. Of these, a three-stage classification was selected as the best one, based on the Akaike information criterion. The presence of both major complication and progressive course (stage 3) was the worst prognostic indicator (43% survival at 15 years). In contrast, no patient died who had neither of these manifestations or who had a progressive course but an elevated ESR as well (stage 1).
The long-term outcome for patients with Takayasu's disease seems best predicted by two major prognostic factors, ie, complications and the pattern of the past clinical course, as well as by ESR. Aggressive medical and surgical treatment may be considered for patients with a major complication and a progressive course (stage 3).
高安氏病是一种病因不明的慢性炎症性动脉病，患者的临床病程各异，长期预后的预测因素尚不清楚。我们基于随访结果研究了该疾病的预后因素，并提出了一种新的预后分类方法。
对120例患者的临床数据应用寿命表法和Cox回归分析，这些患者进行了前瞻性随访，中位随访时间为13年（范围为1个月至34年）。诊断后15年的总生存率为82.9%，在随访期的剩余时间内保持不变。单因素Cox分析显示，在诊断时评估的六个二分变量中，有四个是具有统计学意义的预测因素，包括并发症（高安氏视网膜病变、高血压、主动脉瓣关闭不全和动脉瘤）、既往临床病程模式、患者年龄和诊断年份。因此，有和无主要并发症的患者15年生存率分别为66.3%和96.4%；有和无进展性病程的患者分别为67.9%和92.9%；年龄>35岁和≤35岁的患者分别为58.3%和92.7%；1957年至1975年和1976年至1990年诊断的患者分别为79.9%和96.5%。诊断延迟和红细胞沉降率（ESR）的意义不大。多因素Cox分析显示，上述变量中只有两个是具有统计学意义的独立预测因素，即主要并发症和进展性病程。如果进行Cox逐步剔除程序，ESR是第三个预测因素。这三个预测因素以各种组合使用，理论上总共可进行1822种分类。其中，基于赤池信息准则选择了一个三阶段分类作为最佳分类。同时存在主要并发症和进展性病程（3期）是最差的预后指标（15年生存率为43%）。相比之下，既无这些表现，或有进展性病程但ESR也升高的患者（1期）均无死亡。
高安氏病患者长期预后似乎最好由两个主要预后因素预测，即并发症和既往临床病程模式，以及ESR。对于有主要并发症和进展性病程（3期）的患者，可考虑积极的药物和手术治疗。
