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Original Research: PULMONARY VASCULAR DISEASE |

Clinical Characteristics and Survival in Systemic Sclerosis-Related Pulmonary Hypertension Associated With Interstitial Lung DiseasePulmonary Hypertension in Scleroderma

David Launay, MD, PhD; Marc Humbert, MD, PhD; Alice Berezne, MD; Vincent Cottin, MD, PhD; Yannick Allanore, MD, PhD; Louis-Jean Couderc, MD; Olivier Bletry, MD; Azzedine Yaici, MD; Pierre-Yves Hatron, MD; Luc Mouthon, MD, PhD; Jérôme Le Pavec, MD, PhD; Pierre Clerson, MD; Eric Hachulla, MD, PhD
Author and Funding Information

From the Service de Médecine Interne (Drs Launay, Hatron, and Hachulla), Centre de référence de la sclérodermie, Hôpital Claude-Huriez, CHRU Lille, and Faculté de Médecine (Drs Launay and Le Pavec), d’Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille; Faculté de Médecine (Drs Humbert and Yaici), Université Paris-Sud, Kremlin-Bicêtre; AP-HP, Centre national de référence de l’hypertension pulmonaire sévère (Drs Humbert and Yaici), Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart; INSERM U999 (Dr Humbert), Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson; Université Paris Descartes (Drs Berezne and Mouthon), Pôle de Médecine Interne, Centre de référence des vascularites et de la sclérodermie, Hôpital Cochin, AP-HP, Paris; Hospices Civils de Lyon (Dr Cottin), Service de pneumologie, Centre de référence des maladies pulmonaires rares, Lyon; Université Claude-Bernard Lyon I (Dr Cottin), UMR 754, Lyon; Université Paris Descartes (Dr Allanore), Service de Rhumatologie A, Hôpital Cochin, Paris; Service de Pneumologie (Dr Couderc), and Service de Médecine Interne (Dr Bletry), Hôpital Foch, Suresnes; and Orgamétrie (Dr Clerson), Roubaix, France.

Correspondence to: David Launay, MD, PhD, Service de Médecine Interne, Hôpital Claude-Huriez, CHRU Lille, 1 rue Michel Polonovski, 59037 Lille Cedex, France; e-mail: david.launay@chru-lille.fr


Funding/Support: This study was supported by a research grant from Pfizer.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2011 American College of Chest Physicians


Chest. 2011;140(4):1016-1024. doi:10.1378/chest.10-2473
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Background:  Pulmonary hypertension (PH) complicating systemic sclerosis (SSc)-related interstitial lung disease (ILD) is usually associated with a poor prognosis. However, data are either lacking or scarce on prognostic factors in this condition. The objectives of this study were to compare the survival of patients with ILD-associated PH (PH-ILD) or pulmonary arterial hypertension (PAH) and to determine whether the severity of PH has prognostic value in SSc-associated PH-ILD.

Methods:  Consecutive patients with SSc and PH-ILD (n = 47) or PAH (n = 50) confirmed by right-sided heart catheterization were included in a cross-sectional analysis. PH was classified as mild (mean pulmonary arterial pressure [mPAP] ≤ 35 mm Hg) or moderate to severe (mPAP > 35 mm Hg).

Results:  As compared with patients with PAH, subjects with PH-ILD were younger, were more frequently men with a history of smoking, had more frequently diffuse SSc, less frequently anticentromere antibodies, and a lower FVC/diffusing capacity of lung for carbon monoxide (Dlco) ratio. They had a worse prognosis than patients with PAH (3-year survival of 47% vs 71%, respectively; P = .07). Patients with mild PH-ILD had similar poor outcomes when compared with those with moderate to severe PH-ILD. Pericardial effusion (hazard ratio [HR], 2.44; P = .04) and lower Dlco (HR, 0.96; P = .01) were the only independent factors predictive of a poor survival in the PH-ILD group.

Conclusions:  Patients with SSc with PH-ILD had a different phenotype and a worse prognosis than those with SSc and PAH. Lower Dlco and presence of pericardial effusion were predictive of a poor outcome in PH-ILD, whereas mPAP seemed to have no prognostic significance.

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