Jacobsen S, Halberg P, Ullman S
Department of Rheumatology, Copenhagen University Hospital at Hvidovre, Denmark.
Br J Rheumatol. 1998 Jul;37(7):750-5. doi: 10.1093/rheumatology/37.7.750.
To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement.
A cohort of 344 patients with incident systemic sclerosis (SSc) after 1 January 1960 was retrospectively identified, representing 3716 patient-years of follow-up. The vital status at the end of the study ultimo 1996 was established by reviewing the clinical charts and by contacting the Danish Central Person Register. The causes of death were based on information obtained from the hospital charts and the autopsy reports (80%) and death certificates (20%). The extent of skin sclerosis was described by means of a two-subset model (limited and diffuse involvement) and a three-subset model (digital, extremity and truncal involvement) in all patients. Expected numbers of deaths were calculated by means of age- and sex-specific mortality rates of the general Danish population.
Crude mortality rates were 6.5 and 3.9%/yr, respectively, in men and women. The standardized mortality ratio (SMR) was 2.9 (95% CI 2.5-3.4) and was not significantly influenced by sex or age at disease onset. Mortality rates were significantly increased in patients with diffuse skin sclerosis (SMR 4.5, 95% CI 3.5-5.7) and patients under the age of 35 yr (SMR 13, 95% CI 2.7-37). Of the 160 patients who died during the study, 41 deaths (26%) were SSc related, accounting for one-third of the excess mortality.
We found a significantly increased mortality in patients with SSc, particularly in the subset of patients with diffuse skin sclerosis and in young patients. The excess mortality was due to an increase in both the SSc-related mortality and the unrelated mortality. However, all other subsets also had a significantly increased risk of death, mainly due to an increased risk of death due to causes unrelated to SSc, cancer among others. The fact that the unrelated mortality in SSc was found to be 2-fold increased suggests that the excess mortality that was classified as unrelated should more appropriately be termed indirectly SSc related.
确定丹麦系统性硬化症（硬皮病）患者的生存率、死亡率及死亡原因，并分析这些参数如何受到人口统计学变量和皮肤受累程度的影响。
回顾性确定了1960年1月1日后发病的344例系统性硬化症（SSc）患者队列，随访时间共计3716患者年。通过查阅临床病历并联系丹麦中央人口登记处确定了1996年末研究结束时的生命状态。死亡原因基于从医院病历、尸检报告（80%）和死亡证明（20%）中获取的信息。采用两子集模型（局限性和弥漫性受累）和三子集模型（手指、肢体和躯干受累）描述了所有患者的皮肤硬化程度。通过丹麦普通人群的年龄和性别特异性死亡率计算预期死亡人数。
男性和女性的粗死亡率分别为每年6.5%和3.9%。标准化死亡率（SMR）为2.9（95%可信区间2.5 - 3.4），且不受发病时性别或年龄的显著影响。弥漫性皮肤硬化患者（SMR 4.5，95%可信区间3.5 - 5.7）和35岁以下患者（SMR 13，95%可信区间2.7 - 37）的死亡率显著升高。在研究期间死亡的160例患者中，41例死亡（26%）与SSc相关，占额外死亡率的三分之一。
我们发现SSc患者的死亡率显著升高，尤其是弥漫性皮肤硬化患者子集和年轻患者。额外死亡率是由于与SSc相关的死亡率和非相关死亡率均增加所致。然而，所有其他子集的死亡风险也显著增加，主要是由于与SSc无关的原因导致的死亡风险增加，尤其是癌症。SSc中非相关死亡率增加了2倍这一事实表明，被归类为非相关的额外死亡率更恰当地应称为间接与SSc相关。
