BACKGROUND Systemic sclerosis (SSc) is a severe rheumatic disease of the interstitial tissue, in which heart and lung involvement can lead to disease-specific mortality. Our study tests the hypothesis that in addition to established prognostic factors, cardiopulmonary exercise testing (CPET) parameters, particularly peak oxygen uptake (peakVO2) and ventilation/carbon dioxide (VE/VCO2)-slope, can predict survival in patients with SSc. SUBJECTS AND METHODS We retrospectively assessed 210 patients (80.9% female) in 6 centres over 10 years with pulmonary testing and CPET. Survival was analysed with Cox regression analysis (adjusted for age and gender) by age, comorbidity (Charlson-Index), body weight, body-mass index, extensive interstitial lung disease, pulmonary artery pressure (measured by echocardiography and invasively), and haemodynamic, pulmonary and CPET parameters. RESULTS Five- and ten-year survival of SSc patients was 93.8 and 86.9%, respectively. There was no difference in survival between patients with diffuse (dcSSc) and limited cutaneous manifestation (lcSSc; p = 0.3). Pulmonary and CPET parameters were significantly impaired. Prognosis was worst for patients with pulmonary hypertension (p = 0.007), 6-min walking distance < 413 m (p = 0.003), peakVO2 < 15.6 mL∙kg- 1∙min- 1, and VE/VCO2-slope > 35. Age (hazard ratio HR = 1.23; 95% confidence interval CI: 1.14;1.41), VE/VCO2-slope (HR = 0.9; CI 0.82;0.98), diffusion capacity (Krogh factor, HR = 0.92; CI 0.86;0.98), forced vital capacity (FVC, HR = 0.91; CI 0.86;0.96), and peakVO2 (HR = 0.87; CI 0.81;0.94) were significantly linked to survival in multivariate analyses (Harrell's C = 0.95). This is the first large study with SSc patients that demonstrates the prognostic value of peakVO2 < 15.6 mL∙kg- 1∙min- 1 (< 64.5% of predicted peakVO2) and VE/VCO2-slope > 35.

中文翻译：

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心肺运动测试对系统性硬化症患者的预后价值。

背景技术系统性硬化症（SSc）是间质组织的一种严重的风湿病，其中心脏和肺部受累可导致特定疾病的死亡率。我们的研究检验了以下假设：除确定的预后因素外，心肺运动测试（CPET）参数（尤其是峰值摄氧量（peakVO2）和通气/二氧化碳（VE / VCO2）斜率）还可以预测SSc患者的生存率。研究对象和方法我们回顾性评估了10年内在6个中心进行的210例患者（女性占80.9％）的肺部检查和CPET。使用Cox回归分析法（按年龄和性别调整），根据年龄，合并症（查尔森指数），体重，体质指数，广泛的间质性肺疾病，肺动脉压（通过超声心动图和侵入性测量）和血流动力学分析生存率，肺和CPET参数。结果SSc患者的五年和十年生存率分别为93.8％和86.9％。弥漫性（dcSSc）和皮肤表现受限（lcSSc； p = 0.3）的患者的生存率无差异。肺和CPET参数明显受损。对于肺动脉高压（p = 0.007），6分钟步行距离<413 m（p = 0.003），峰值VO2 <15.6 mL∙kg-1∙min-1和VE / VCO2-斜率> 35的患者，预后最差。年龄（危险比HR = 1.23; 95％置信区间CI：1.14; 1.41），VE / VCO2斜率（HR = 0.9; CI 0.82; 0.98），扩散能力（Krogh因子，HR = 0.92; CI 0.86; 0.98）在多变量分析中，强迫肺活量（FVC，HR = 0.91; CI 0.86; 0.96）和峰值VO2（HR = 0.87; CI 0.81; 0.94）与生存率显着相关（Harrell's C = 0.95）。