Czirják L, Kumánovics G, Varjú C, Nagy Z, Pákozdi A, Szekanecz Z, Szucs G
University of Pécs, Medical School, Department of Immunology and Rheumatology, H-7621 Pécs, Hungary.
Ann Rheum Dis. 2008 Jan;67(1):59-63. doi: 10.1136/ard.2006.066340. Epub 2007 May 22.
Survival analysis of a series of 366 consecutive patients with systemic sclerosis (SSc).
Clinical and laboratory data were evaluated from 1983 until 2005 using a standard protocol. The female/male ratio was 315/51. The mean (SD) age of the patients was 56.8 (12.2) years. The duration of disease was 12 (5-19) years with a median follow-up of 6 (3-12) years.
Kaplan-Meier univariate analysis showed that renal, cardiac involvement, pigmentation disturbances, malabsorption, a forced vital capacity <50%, diffuse scleroderma, presence of early malignancy, anaemia, and increased erythrocyte sedimentation rate (ESR) were signs of unfavourable prognosis, whereas anti-centromere antibodies were indicators of a good survival. In the multivariate Cox proportional hazards model the presence of diffuse scleroderma, renal involvement, coexistence of a malignant disease, and increased ESR were poor independent prognostic signs. Elderly age at the onset of disease also caused an unfavourable outcome. A total of 86 SSc-related deaths were recorded during the follow-up. Of them, 65% were attributed to cardiorespiratory manifestation of disease. Tumour associated early death was found in 12 cases (14%).
In addition to the well-known factors influencing the outcome (diffuse subset, internal organ involvements, and inflammatory signs), the coexistence of scleroderma with a malignancy also causes a poor outcome.
对连续366例系统性硬化症（SSc）患者进行生存分析。
采用标准方案对1983年至2005年期间的临床和实验室数据进行评估。女性/男性比例为315/51。患者的平均（标准差）年龄为56.8（12.2）岁。病程为12（5 - 19）年，中位随访时间为6（3 - 12）年。
Kaplan - Meier单因素分析显示，肾脏、心脏受累、色素沉着紊乱、吸收不良、用力肺活量<50%、弥漫性硬皮病、早期恶性肿瘤、贫血以及红细胞沉降率（ESR）升高是预后不良的征象，而抗着丝点抗体是生存良好的指标。在多变量Cox比例风险模型中，弥漫性硬皮病、肾脏受累、恶性疾病并存以及ESR升高是不良的独立预后征象。疾病发病时年龄较大也导致不良结局。随访期间共记录了86例与SSc相关的死亡病例。其中，65%归因于疾病的心肺表现。12例（14%）发现与肿瘤相关的早期死亡。
除了影响预后的众所周知的因素（弥漫性亚型、内脏器官受累以及炎症征象）外，硬皮病与恶性肿瘤并存也会导致不良结局。
