Nagy Gabriella, Minier Tünde, Varjú Cecília, Faludi Réka, Kovács Katalin T, Lóránd Veronika, Hermann Veronika, Czirják László, Kumánovics Gábor
Department of Rheumatology and Immunology, University of Pécs, Hungary.
Heart Institute, Medical Center University of Pécs, Hungary.
Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):61-70. Epub 2017 Aug 31.
Analysis of risk factors and mortality of 439 patients with systemic sclerosis (SSc) in a tertiary care centre.
The mean follow up time was 8.4±5.6 years. Lost to follow up rate was 6.4%. Female to male ratio was 366 to 73. Two hundred sixty patients had limited and 179 diffuse cutaneous SSc (dcSSc). A standard protocol including musculoskeletal examinations was used for the assessment of patients.
By Kaplan-Meier analysis the overall 5-, 10- and 15 year survival were 88.2%, 79.9% and 73.6%, respectively. Univariate analysis showed that dcSSc, male gender, presence of small joint contractures, pulmonary interstitial, cardiac, oesophageal involvement, scleroderma renal crisis, arterial hypertension, anti-topoisomerase antibody, anemia, hypalbuminemia, coexistent malignancies and elevated erythrocyte sedimentation were associated with poor survival. Lack of giant capillaries, avascular zones or neo-angiogenesis on capillaroscopy, and presence of anti-centromere antibodies were associated with favourable outcome. Multivariate regression analysis showed presence of small joint contractures, history of arterial hypertension, male gender, diffusing capacity of carbon monoxide <50%, right ventricular pressure >40 mmHg on echocardiography, less than 50% ejection fraction, anti-topoisomerase I positivity, anemia, and serum albumin concentration < 35 g/l as well as current or history of coexistent malignancy were independent poor prognostic factors.
In addition to well-known factors predicting poor outcome in SSc, the presence of small joint contractures was a newly identi ed independent risk factor of mortality. Our data also confirmed a recent finding showing that history of arterial hypertension was also a poor prognostic factor.
分析一家三级医疗中心439例系统性硬化症（SSc）患者的危险因素及死亡率。
平均随访时间为8.4±5.6年。失访率为6.4%。男女比例为366比73。260例患者为局限性皮肤型SSc，179例为弥漫性皮肤型SSc（dcSSc）。采用包括肌肉骨骼检查在内的标准方案对患者进行评估。
通过Kaplan-Meier分析，总体5年、10年和15年生存率分别为88.2%、79.9%和73.6%。单因素分析显示，dcSSc、男性、小关节挛缩、肺间质病变、心脏病变、食管受累、硬皮病肾危象、动脉高血压、抗拓扑异构酶抗体、贫血、低白蛋白血症、并存恶性肿瘤以及红细胞沉降率升高与生存率低相关。毛细血管镜检查显示缺乏巨大毛细血管、无血管区或新生血管形成，以及存在抗着丝点抗体与良好预后相关。多因素回归分析显示，小关节挛缩、动脉高血压病史、男性、一氧化碳弥散量<50%、超声心动图显示右心室压力>40 mmHg、射血分数<50%、抗拓扑异构酶I阳性、贫血、血清白蛋白浓度<35 g/l以及并存恶性肿瘤的现患或既往史是独立的不良预后因素。
除了已知的预测SSc不良预后的因素外，小关节挛缩的存在是新发现的独立死亡危险因素。我们的数据还证实了最近的一项发现，即动脉高血压病史也是不良预后因素。
