developing pulmonary vasculopathy in systemic sclerosis, detected with non-invasive cardiopulmonary exercise testing发展中肺血管病变系统性硬化症,发现与非侵入性心肺运动试验.pdfVIP

Developing Pulmonary Vasculopathy in Systemic Sclerosis, Detected with Non-Invasive Cardiopulmonary Exercise Testing Daniel Dumitrescu1,2,5*, Ronald J. Oudiz1,3, George Karpouzas 1,4, Arsen Hovanesyan1,3, Amali 1,2 1,2 5 1,2 Jayasinghe , James E. Hansen , Stephan Rosenkranz , Karlman Wasserman 1 Los Angeles Biomedical Research Institute, Harbor-UCLA Medical Center, Torrance, California, United States of America, 2 Division of Respiratory and Critical Care Medicine and Physiology, Klinik III fuer Innere Medizin, Herzzentrum der Universitaet zu Koeln, Cologne, Germany, 3 Division of Cardiology, Klinik III fuer Innere Medizin, Herzzentrum der Universitaet zu Koeln, Cologne, Germany, 4 Division of Rheumatology, Klinik III fuer Innere Medizin, Herzzentrum der Universitaet zu Koeln, Cologne, Germany, 5 Klinik III fuer Innere Medizin, Herzzentrum der Universitaet zu Koeln, Cologne, Germany Abstract Background: Patients with systemic sclerosis (SSc) may develop exercise intolerance due to musculoskeletal involvement, restrictive lung disease, left ventricular dysfunction, or pulmonary vasculopathy (PV). The latter is particularly important since it may lead to lethal pulmonary arterial hypertension (PAH). We hypothesized that abnormalities during cardiopulmonary exercise testing (CPET) in patients with SSc can identify PV leading to overt PAH. Methods: Thirty SSc patients from the Harbor-UCLA Rheumatology clinic, not clinically suspected of having significant pulmonary vascular disease, were referred for this prospective study. Resting pulmonary function and exercise gas