Ewert, Ralf and Ittermann, Till and Habedank, Dirk and Held, Matthias and Lange, Tobias J. and Halank, Michael and Winkler, Joerg and Glaeser, Sven and Olschewski, Horst and Kovacs, Gabor (2019) Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis. BMC PULMONARY MEDICINE, 19 (1): 230. ISSN 1471-2466,
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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 (peakVO(2)) 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), peakVO(2) < 15.6 mL center dot kg(- 1)center dot 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 peakVO(2) (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 peakVO(2) < 15.6 mL center dot kg(- 1)center dot min(- 1) (< 64.5% of predicted peakVO(2)) and VE/VCO2-slope > 35.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | PULMONARY ARTERIAL-HYPERTENSION; INTERSTITIAL LUNG-DISEASE; RIGHT-HEART CATHETERIZATION; 6-MINUTE WALK TEST; CLINICAL CHARACTERISTICS; MYCOPHENOLATE-MOFETIL; SCLERODERMA LUNG; DELPHI CONSENSUS; SURVIVAL; MORTALITY; Systemic sclerosis; Cardiopulmonary exercise; Pulmonary function; Prognosis; Pulmonary hypertension |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Innere Medizin II |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 24 Mar 2020 07:46 |
| Last Modified: | 24 Mar 2020 07:46 |
| URI: | https://pred.uni-regensburg.de/id/eprint/25794 |
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