Nihal Martis1,2, Viviane Queyrel-Moranne3,4, David Launay3,4, Rémi Neviere3,4, Jean-Gabriel Fuzibet3,4, Charles-Hugo Marquette3,4, Sylvie Leroy3,4. 1. From the Service de Médecine Interne, Centre Hospitalier Universitaire (CHU) de Nice, Nice; University of Lille, U995 Lille Inflammation Research International Center (LIRIC), Lille; INSERM, U995, Lille; CHU Lille, Département de Médecine Interne et Immunologie Clinique, Lille; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), Lille; Service d'Explorations Fonctionnelles Respiratoires, CHU de Lille, Lille; FHU OncoAge, Côte d'Azur University, Nice, France. martis.n@chu-nice.fr. 2. N. Martis, MD, MS, Service de Médecine Interne, CHU de Nice; V. Queyrel-Moranne, MD, MS, Service de Médecine Interne, CHU de Nice; D. Launay, MD, PhD, Professor of Medicine, Univ. Lille, U995, LIRIC, INSERM U995, CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique); R. Neviere, MD, PhD, Professor of Medicine, Service d'Explorations Fonctionnelles Respiratoires, CHU de Lille; J.G. Fuzibet, MD, MS, Professor of Medicine, Service de Médecine Interne, CHU de Nice; C.H. Marquette, MD, PhD, Professor of Medicine, FHU OncoAge, Côte d'Azur University; S. Leroy, MD, FHU OncoAge, Côte d'Azur University. martis.n@chu-nice.fr. 3. From the Service de Médecine Interne, Centre Hospitalier Universitaire (CHU) de Nice, Nice; University of Lille, U995 Lille Inflammation Research International Center (LIRIC), Lille; INSERM, U995, Lille; CHU Lille, Département de Médecine Interne et Immunologie Clinique, Lille; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), Lille; Service d'Explorations Fonctionnelles Respiratoires, CHU de Lille, Lille; FHU OncoAge, Côte d'Azur University, Nice, France. 4. N. Martis, MD, MS, Service de Médecine Interne, CHU de Nice; V. Queyrel-Moranne, MD, MS, Service de Médecine Interne, CHU de Nice; D. Launay, MD, PhD, Professor of Medicine, Univ. Lille, U995, LIRIC, INSERM U995, CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique); R. Neviere, MD, PhD, Professor of Medicine, Service d'Explorations Fonctionnelles Respiratoires, CHU de Lille; J.G. Fuzibet, MD, MS, Professor of Medicine, Service de Médecine Interne, CHU de Nice; C.H. Marquette, MD, PhD, Professor of Medicine, FHU OncoAge, Côte d'Azur University; S. Leroy, MD, FHU OncoAge, Côte d'Azur University.
Abstract
OBJECTIVE: Exercise limitation in patients with systemic sclerosis (SSc) is often multifactorial and related to complications such as interstitial lung disease (ILD), pulmonary vasculopathy (PV), left ventricular dysfunction (LVD), and/or peripheral/muscular limitation (PML). We hypothesized that cardiopulmonary exercise testing (CPET) could not only suggest and rank competing etiologies, but also highlight peripheral impairment. METHODS: Clinical, resting pulmonary function testing, and CPET data from patients with SSc referred for exercise limitation between October 2009 and November 2015 were retrospectively analyzed in this bi-center study. Patients were categorized as having ILD, PV, LVD, and/or PML based on CPET response patterns and the diagnoses were matched with results from the reference investigations. The latter consisted of transthoracic echocardiography, chest computed tomography scan, and right heart catheterization (RHC). RESULTS: Twenty-seven patients presented with CPET profiles consistent with ILD (n = 16), PV (n = 15), LVD (n = 5), and PML (n = 19). None of the subjects had a normal CPET profile. There was a statistically significant negative correlation between resting DLCO, on the one hand, and dead space to tidal volume ratio and alveolar-arterial gradient [P(Ai-a)O2] on the other (p < 0.005). CPET identified 90% of patients with a mean pulmonary arterial pressure at rest ≥ 21 mmHg measured by RHC (n = 10). Peak P(Ai-a)O2, taken independently from other variables, was crucial in distinguishing subjects with ILD from those without ILD (p < 0.05). CONCLUSION: CPET is useful for the characterization of multifactorial exercise limitation in patients with SSc and in identifying SSc-related complications such as ILD and PV. This study also identifies PML as an underestimated cause of exercise limitation.
OBJECTIVE: Exercise limitation in patients with systemic sclerosis (SSc) is often multifactorial and related to complications such as interstitial lung disease (ILD), pulmonary vasculopathy (PV), left ventricular dysfunction (LVD), and/or peripheral/muscular limitation (PML). We hypothesized that cardiopulmonary exercise testing (CPET) could not only suggest and rank competing etiologies, but also highlight peripheral impairment. METHODS: Clinical, resting pulmonary function testing, and CPET data from patients with SSc referred for exercise limitation between October 2009 and November 2015 were retrospectively analyzed in this bi-center study. Patients were categorized as having ILD, PV, LVD, and/or PML based on CPET response patterns and the diagnoses were matched with results from the reference investigations. The latter consisted of transthoracic echocardiography, chest computed tomography scan, and right heart catheterization (RHC). RESULTS: Twenty-seven patients presented with CPET profiles consistent with ILD (n = 16), PV (n = 15), LVD (n = 5), and PML (n = 19). None of the subjects had a normal CPET profile. There was a statistically significant negative correlation between resting DLCO, on the one hand, and dead space to tidal volume ratio and alveolar-arterial gradient [P(Ai-a)O2] on the other (p < 0.005). CPET identified 90% of patients with a mean pulmonary arterial pressure at rest ≥ 21 mmHg measured by RHC (n = 10). Peak P(Ai-a)O2, taken independently from other variables, was crucial in distinguishing subjects with ILD from those without ILD (p < 0.05). CONCLUSION: CPET is useful for the characterization of multifactorial exercise limitation in patients with SSc and in identifying SSc-related complications such as ILD and PV. This study also identifies PML as an underestimated cause of exercise limitation.
Authors: Cristina Antinozzi; Elisa Grazioli; Maria De Santis; Francesca Motta; Paolo Sgrò; Federico Mari; Caterina Mauri; Attilio Parisi; Daniela Caporossi; Guglielmo Duranti; Roberta Ceci; Luigi Di Luigi; Ivan Dimauro Journal: Int J Environ Res Public Health Date: 2022-08-18 Impact factor: 4.614