David Launay1, Marc Humbert2, Alice Berezne3, Vincent Cottin4, Yannick Allanore5, Louis-Jean Couderc6, Olivier Bletry7, Azzedine Yaici8, Pierre-Yves Hatron9, Luc Mouthon3, Jérôme Le Pavec10, Pierre Clerson11, Eric Hachulla9. 1. Service de Médecine Interne, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille; Centre de référence de la sclérodermie, Hôpital Claude-Huriez, CHRU Lille, Faculté de Médecine, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille. Electronic address: david.launay@chru-lille.fr. 2. Faculté de Médecine, Université Paris-Sud, Kremlin-Bicêtre; AP-HP, Centre national de référence de l'hypertension pulmonaire sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart; INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson. 3. Université Paris Descartes, Pôle de Médecine Interne, Centre de référence des vascularites et de la sclérodermie, Hôpital Cochin, AP-HP, Paris. 4. Hospices Civils de Lyon, Service de pneumologie, Centre de référence des maladies pulmonaires rares, Lyon; Université Claude-Bernard Lyon I, UMR 754, Lyon. 5. Université Paris Descartes, Service de Rhumatologie A, Hôpital Cochin, Paris. 6. Service de Pneumologie, Hôpital Foch, Suresnes. 7. Service de Médecine Interne, Hôpital Foch, Suresnes. 8. Faculté de Médecine, Université Paris-Sud, Kremlin-Bicêtre; AP-HP, Centre national de référence de l'hypertension pulmonaire sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart. 9. Service de Médecine Interne, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille. 10. Centre de référence de la sclérodermie, Hôpital Claude-Huriez, CHRU Lille, Faculté de Médecine, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille. 11. Orgamétrie, Roubaix, France.
Abstract
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.
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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