Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease

Autor: Gilbert Habib, Fabien Subtil, Laurent Bertoletti, Marc Humbert, Judith Catella-Chatron, Julie Traclet, K. Ahmad, David Montani, Jean-François Mornex, Vincent Cottin, D. Gamondes, David Launay, Sébastien Renard, Ségolène Turquier, Boubou Camara, Sabrina Zeghmar, Anne-Marie Schott, Julien Berthiller, Ana Nieves, Emmanuel Gomez, Olivier Sanchez, Louis Chauvelot, Grégoire Prévot, Martine Reynaud-Gaubert
Přispěvatelé: Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Hôpital Nord AP‐MM Marseille, France (AP‐MM Marseille), Hôpital de la Timone [CHU - APHM] (TIMONE), Santé Ingénierie Biologie Saint-Etienne (SAINBIOSE), Centre Ingénierie et Santé (CIS-ENSMSE), École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Clinique Universitaire Pneumologique, Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, CHU Grenoble, Centre Hospitalier Universitaire de Nancy (CHU Nancy), CHU Lille, Hôpital Bicêtre, Université Paris-Saclay, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)
Rok vydání: 2019
Předmět:
Endothelin Receptor Antagonists
Male
Cardiac Catheterization
Haemodynamic response
Vital Capacity
Hemodynamics
0302 clinical medicine
Interquartile range
Diffusing capacity
Forced Expiratory Volume
Immunology and Allergy
skin and connective tissue diseases
Pulmonary Arterial Hypertension
integumentary system
Interstitial lung disease
respiratory system
Middle Aged
Prognosis
Survival Rate
medicine.anatomical_structure
Treatment Outcome
[SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system
Cardiology
Female
Immunosuppressive Agents
medicine.medical_specialty
Hypertension
Pulmonary
Immunology
Walk Test
03 medical and health sciences
Rheumatology
Internal medicine
medicine
Humans
Aged
030203 arthritis & rheumatology
Scleroderma
Systemic
business.industry
Oxygen Inhalation Therapy
Phosphodiesterase 5 Inhibitors
medicine.disease
Pulmonary hypertension
Epoprostenol
030228 respiratory system
Heart catheterization
Vascular resistance
Pulmonary Diffusing Capacity
business
Lung Diseases
Interstitial
Zdroj: Arthritis & rheumatology
Arthritis & rheumatology, 2021, 73 (2), pp.295-304. ⟨10.1002/art.41512⟩
ISSN: 2326-5205
2326-5191
Popis: International audience; Objective Patients with systemic sclerosis and both pulmonary hypertension and interstitial lung disease (SSc-PH-ILD) generally carry a worse prognosis than patients with SSc and pulmonary arterial hypertension (SSc-PAH) without ILD. There is no evidence of the efficacy of PAH therapies in SSc-PH-ILD. We undertook this study to compare survival of and response to treatment in patients with SSc-PH-ILD and those with SSc-PAH.Methods We analyzed 128 patients (66 with SSc-PH-ILD and 62 with SSc-PAH) from 15 centers, in whom PH was diagnosed by right-sided heart catheterization; they were prospectively included in the PH registry. All patients received PAH-specific therapy. Computed tomography of the chest was used to confirm or exclude ILD.Results At baseline, patients with SSc-PH-ILD had less severe hemodynamic impairment than those with SSc-PAH (pulmonary vascular resistance 5.7 Wood units versus 8.7 Wood units; P = 0.0005) and lower diffusing capacity for carbon monoxide (median 25% [interquartile range (IQR) 18%, 35%] versus 40% [IQR 31%, 51%]; P = 0.0005). Additionally, patients with SSc-PH-ILD had increased mortality (8.1% at 1 year, 21.2% at 2 years, and 41.5% at 3 years) compared to those with SSc-PAH (4.1%, 8.7%, and 21.4%, respectively; P = 0.04). Upon treatment with PAH-targeted therapy, no improvement in the 6-minute walk distance was observed in either group. Improvement in the World Health Organization functional class was observed less frequently in patients with SSc-ILD-PH compared to those with SSc-PAH (13.6% versus 33.3%; P = 0.02). Hemodynamics improved similarly in both groups.Conclusion ILD confers a worse prognosis to SSc-PH. Response to PAH-specific therapy is clinically poor in SSc-PH-ILD but was not found to be hemodynamically different from the response observed in SSc-PAH.
Databáze: OpenAIRE
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