Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis.
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ABSTRACT: 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 ?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??35.
SUBMITTER: Ewert R
PROVIDER: S-EPMC6884803 | biostudies-literature | 2019 Nov
REPOSITORIES: biostudies-literature
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