Department of Rheumatology, Oslo University Hospital - Rikshospitalet, Norway.
J Rheumatol. 2013 Jul;40(7):1127-33. doi: 10.3899/jrheum.121390. Epub 2013 May 1.
To determine survival and causes of death in an unselected and complete cohort of Norwegian patients with systemic sclerosis (SSc) compared to the background population.
Multiple methods were used to identify every patient with SSc living in southeast Norway, with a denominator population of 2,707,012, between 1999 and 2009. All patients who met either the American College of Rheumatology criteria or the Medsger and LeRoy criteria for SSc were included. Every patient was matched for sex and age with 15 healthy controls drawn from the national population registry. Vital status at January 1, 2010, was provided for patients and controls by the national population registry. Causes of death were obtained from death certificates and by chart review.
Forty-three (14%) of 312 patients with SSc died during the study period. The standardized mortality rate (SMR) was estimated to be 2.03 for the entire cohort and 5.33 for the subgroup with diffuse cutaneous (dc) SSc. The 5- and 10-year survival rates were 91% and 70%, respectively, for dcSSc and 98% and 93% for limited cutaneous (lc) SSc. Causes of death were related to SSc in 24/43 (56%) patients, mostly cardiopulmonary diseases (n = 13), including pulmonary hypertension (n = 8). Factors associated with fatal outcome included male sex, dcSSc, pulmonary hypertension, and interstitial lung disease.
Compared to the Norwegian background population, our cohort of 312 unselected patients with SSc had decreased survival. The survival rates observed were, however, better than those previously reported from SSc referral centers.
与背景人群相比，确定挪威系统性硬化症（SSc）未选择和完整队列患者的生存和死亡原因。
使用多种方法确定了 1999 年至 2009 年间生活在挪威东南部的每一位患有 SSc 的患者，其分母人群为 2707012 人。所有符合美国风湿病学会标准或 Medsger 和 LeRoy 标准的 SSc 患者均被纳入研究。每位患者均按性别和年龄与从全国人口登记处抽取的 15 名健康对照者相匹配。全国人口登记处为患者和对照者提供了截至 2010 年 1 月 1 日的生存状态。通过死亡证明和病历回顾获得死亡原因。
在研究期间，312 例 SSc 患者中有 43 例（14%）死亡。整个队列的标准化死亡率（SMR）估计为 2.03，弥漫性皮肤型（dc）SSc 亚组为 5.33。dcSSc 的 5 年和 10 年生存率分别为 91%和 70%，lcSSc 分别为 98%和 93%。43 例（56%）死亡患者的死亡原因与 SSc 有关，主要为心肺疾病（n=13），包括肺动脉高压（n=8）。与致命结局相关的因素包括男性、dcSSc、肺动脉高压和间质性肺病。
与挪威背景人群相比，我们的 312 例未选择的 SSc 患者队列的生存率较低。然而，观察到的生存率优于以前从 SSc 转诊中心报告的生存率。
