Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

BMC Pulmonary Medicine

Dirk Habedank¹  Michael Halank²  Tobias J. Lange³  Horst Olschewski⁴  Gabor Kovacs⁴  Ralf Ewert⁵  Sven Gläser⁶  Till Ittermann⁷  Matthias Held⁸  Jörg Winkler⁹ 

[1] Department of Cardiology, DRK Kliniken Berlin;Department of Internal Medicine I, University Hospital of TU Dresden;Department of Internal Medicine II, University Medical Center Regensburg;Department of Internal Medicine, Medical University of Graz, Division of Pulmonology;Department of Internal Medicine, University Greifswald;Department of Internal Medicine, Vivantes Kliniken Berlin;Institute for Community Medicine, University Greifswald;Missio Clinic;PSL Pulmonary Study Center;

关键词: Systemic sclerosis;    Cardiopulmonary exercise;    Pulmonary function;    Prognosis;    Pulmonary hypertension;   

DOI  :  10.1186/s12890-019-1003-7

来源: DOAJ

【 摘 要 】

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 < 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). Summary 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.

【 授权许可】

Unknown