Altman R D, Medsger T A, Bloch D A, Michel B A
Department of Medicine, University of Miami School of Medicine, FL 33101.
Arthritis Rheum. 1991 Apr;34(4):403-13. doi: 10.1002/art.1780340405.
We conducted followup of 264 patients with definite systemic sclerosis (SSc) who were entered into the multicenter Scleroderma Criteria Cooperative Study (SCCS) during 1973-1977. At the end of the study (average 5.2 years of followup), 38% were known to be alive, 50% were dead (68% of these deaths definitely related to SSc), and 12% were lost to followup. Survival analyses of 484 demographic, clinical, and laboratory items recorded at entry into the SCCS (within 2 years of physician diagnosis of SSc) were performed. Survival declined linearly, and the cumulative survival rate was less than 80% at 2 years, 50% at 8.5 years, and 30% at 12 years after entry. Analysis using combinations of entry variables identifying organ system involvement confirmed that renal, cardiac, pulmonary, and gastrointestinal involvement in SSc predicted reduced survival; however, data on organ system involvement at study entry could not be used to consistently predict which organ system would ultimately be involved as the primary cause of death. By survival tree analysis, the individual entry variables best predicting reduced survival included older age (greater than 64 years), reduced renal function (blood urea nitrogen greater than 16 mg/dl), anemia (hemoglobin less than or equal to 11 gm/dl), reduced pulmonary diffusing capacity for carbon monoxide (less than or equal to 50% of predicted), reduced total serum protein level (less than or equal to 6 gm/dl), and reduced pulmonary reserve (forced vital capacity less than 80% with hemoglobin greater than 14 gm/dl or forced vital capacity less than 65% with hemoglobin less than or equal to 14 gm/dl). Cox proportional hazards model analysis confirmed these results. Different combinations of variables led to markedly different survival rates. The poorest prospects for survival were in patients with SSc who were less than or equal to 64 years old with a hemoglobin level less than or equal to 11 gm/dl, and in those greater than 64 years old with a blood urea nitrogen level greater than 16 mg/dl. These results may be useful in predicting individual patients at risk for shortened survival.
我们对1973年至1977年期间纳入多中心硬皮病标准合作研究（SCCS）的264例确诊系统性硬化症（SSc）患者进行了随访。研究结束时（平均随访5.2年），已知38%的患者存活，50%的患者死亡（其中68%的死亡明确与SSc相关），12%的患者失访。对SCCS入组时（医生诊断SSc后2年内）记录的484项人口统计学、临床和实验室指标进行了生存分析。生存率呈线性下降，入组后2年累积生存率低于80%，8.5年时为50%，12年时为30%。使用确定器官系统受累的入组变量组合进行分析证实，SSc患者的肾脏、心脏、肺部和胃肠道受累预示生存率降低；然而，研究入组时器官系统受累的数据不能用于一致地预测哪个器官系统最终会成为主要死亡原因。通过生存树分析，最能预测生存率降低的个体入组变量包括年龄较大（大于64岁）、肾功能降低（血尿素氮大于16 mg/dl）、贫血（血红蛋白小于或等于11 gm/dl）、一氧化碳肺弥散能力降低（小于或等于预测值的50%）、血清总蛋白水平降低（小于或等于6 gm/dl）以及肺储备降低（血红蛋白大于14 gm/dl时用力肺活量小于80%或血红蛋白小于或等于14 gm/dl时用力肺活量小于65%）。Cox比例风险模型分析证实了这些结果。不同的变量组合导致明显不同的生存率。生存前景最差的是血红蛋白水平小于或等于11 gm/dl且年龄小于或等于64岁的SSc患者，以及血尿素氮水平大于16 mg/dl且年龄大于64岁的患者。这些结果可能有助于预测个体患者生存缩短的风险。
