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: Chauvelot L; Hospices Civils de Lyon, Centre de Référence National des Maladies Pulmonaires Rares, Centre de Compétence de l'Hypertension Pulmonaire, Hôpital Louis Pradel, UMR 754, Université Claude Bernard Lyon 1, OrphaLung, RespiFil, and ERN-LUNG, Lyon, France., Gamondes D; Hospices Civils de Lyon and Hôpital Louis Pradel, Lyon, France., Berthiller J; Hospices Civils de Lyon, Université Claude Bernard Lyon 1, and Health Services and Performance Research (HESPER) EA7425, Lyon, France., Nieves A; Centre de Compétences des Maladies Pulmonaires Rares, and Hôpital Nord, AP-HM, Marseille, France., Renard S; Hôpital de la Timone, AP-HM, Marseille, France., Catella-Chatron J; INSERM U1059 and CHU de Saint-Etienne, Saint-Etienne, France., Ahmad K; Hospices Civils de Lyon, Centre de Référence National des Maladies Pulmonaires Rares, Centre de Compétence de l'Hypertension Pulmonaire, Hôpital Louis Pradel, UMR 754, Université Claude Bernard Lyon 1, OrphaLung, RespiFil, and ERN-LUNG, Lyon, France., Bertoletti L; INSERM U1059 and CHU de Saint-Etienne, Saint-Etienne, France., Camara B; Clinique Universitaire de Pneumologie and CHU de Grenoble Alpes, Grenoble, France., Gomez E; CHRU de Nancy, Vandœuvre-lès-Nancy, France., Launay D; Université de Lille, CHU de Lille, and Centre de Référence des Maladies Systémiques et Auto-Immunes Rares, Lille, France., Montani D; Hôpital Bicêtre, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, INSERM U999, Université Paris-Saclay, Paris, France., Mornex JF; Hospices Civils de Lyon, Centre de Référence National des Maladies Pulmonaires Rares, Centre de Compétence de l'Hypertension Pulmonaire, Hôpital Louis Pradel, UMR 754, Université Claude Bernard Lyon 1, OrphaLung, RespiFil, and ERN-LUNG, Lyon, France., Prévot G; Centre de Compétences des Maladies Pulmonaires Rares, CHU de Toulouse, and Hôpital Larrey, Toulouse, France., Sanchez O; Hôpital Européen Georges Pompidou, AP-HP, Centre de Compétences des Maladies Pulmonaires Rares, Université Paris Descartes, INSERM U1140, Paris, France., Schott AM; Centre de Compétences des Maladies Pulmonaires Rares, and Hôpital Nord, AP-HM, Marseille, France., Subtil F; Université de Lyon, Université Claude Bernard Lyon 1, CNRS UMR 5558, Villeurbanne, France, and Hospices Civils de Lyon, Lyon, France., Traclet J; Hospices Civils de Lyon, Centre de Référence National des Maladies Pulmonaires Rares, Centre de Compétence de l'Hypertension Pulmonaire, Hôpital Louis Pradel, UMR 754, Université Claude Bernard Lyon 1, OrphaLung, RespiFil, and ERN-LUNG, Lyon, France., Turquier S; Hospices Civils de Lyon, Centre de Référence National des Maladies Pulmonaires Rares, Centre de Compétence de l'Hypertension Pulmonaire, Hôpital Louis Pradel, UMR 754, Université Claude Bernard Lyon 1, OrphaLung, RespiFil, and ERN-LUNG, Lyon, France., Zeghmar S; Hospices Civils de Lyon, Centre de Référence National des Maladies Pulmonaires Rares, Centre de Compétence de l'Hypertension Pulmonaire, Hôpital Louis Pradel, UMR 754, Université Claude Bernard Lyon 1, OrphaLung, RespiFil, and ERN-LUNG, Lyon, France., Habib G; Hôpital de la Timone, AP-HM, Marseille, France., Reynaud-Gaubert M; Centre de Compétences des Maladies Pulmonaires Rares, and Hôpital Nord, AP-HM, Marseille, France., Humbert M; Hôpital Bicêtre, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, INSERM U999, Université Paris-Saclay, Paris, France., Cottin V; Hospices Civils de Lyon, Centre de Référence National des Maladies Pulmonaires Rares, Centre de Compétence de l'Hypertension Pulmonaire, Hôpital Louis Pradel, UMR 754, Université Claude Bernard Lyon 1, OrphaLung, RespiFil, and ERN-LUNG, Lyon, France.
Jazyk: angličtina
Zdroj: Arthritis & rheumatology (Hoboken, N.J.) [Arthritis Rheumatol] 2021 Feb; Vol. 73 (2), pp. 295-304. Date of Electronic Publication: 2020 Dec 29.
DOI: 10.1002/art.41512
Abstrakt: 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.
(© 2020, American College of Rheumatology.)
Databáze: MEDLINE
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