Eason R J, Tan P L, Gow P J
Aust N Z J Med. 1981 Dec;11(6):657-62. doi: 10.1111/j.1445-5994.1981.tb03542.x.
The clinical records of all patients with scleroderma or Raynaud's phenomenon who attended hospitals or specialist practice in Auckland during the ten year period of 1970--79 were examined. Forty-seven patients were found to satisfy the American Rheumatism Association criteria for progressive systemic sclerosis (PSS). Thirteen of these patients had CRST features which comprise calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasiae. All but two of the 23 surviving patients were interviewed. The incidence of PSS was 6.3/million/year and no significant difference in incidence was found between Caucasians and Polynesians. Raynaud's phenomenon was most frequently the presenting symptom. Oesophageal involvement was the commonest clinical visceral manifestation. Cumulative survival rates showed that the adverse prognostic features were renal, cardiac and to a lesser extent pulmonary involvement. Patients with the CRST features had a better prognosis and none had renal complications. The natural history of the CRST group was characterised by a long latency between the presenting symptom and the other features of the disease. Therefore, the recognition of the CRST variant was of retrospective rather than predictive clinical value. In this first clinical study of PSS patients in New Zealand, results of therapeutic intervention were disappointing.
对1970年至1979年这十年间在奥克兰各医院或专科诊所就诊的所有硬皮病或雷诺现象患者的临床记录进行了检查。发现有47名患者符合美国风湿病协会的进行性系统性硬化症（PSS）标准。其中13名患者具有CRST特征，包括钙质沉着、雷诺现象、指（趾）硬皮病和毛细血管扩张。在23名存活患者中，除两名外，其余均接受了访谈。PSS的发病率为每年6.3/百万，白种人和波利尼西亚人之间的发病率没有显著差异。雷诺现象最常为首发症状。食管受累是最常见的临床内脏表现。累积生存率表明，不良预后特征为肾脏、心脏受累，肺部受累程度较轻。具有CRST特征的患者预后较好，且均无肾脏并发症。CRST组的自然病史特点是首发症状与疾病的其他特征之间存在较长的潜伏期。因此，CRST变异型的识别具有回顾性而非预测性的临床价值。在新西兰对PSS患者进行的首次临床研究中，治疗干预的结果令人失望。
