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Article Dans Une Revue Chest Année : 2011

Clinical characteristics and survival in systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease

Jérôme Le Pavec
Pierre Clerson
  • Fonction : Auteur
Eric Hachulla

Résumé

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 rightsided 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 anti-centromere 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. CHEST 2011; 140(4):1016-1024
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Dates et versions

hal-02650265 , version 1 (29-05-2020)

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David Launay, Marc Humbert, Alice Berezne, Vincent Cottin, Yannick Allanore, et al.. Clinical characteristics and survival in systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease. Chest, 2011, 140 (4), pp.1016-1024. ⟨10.1378/chest.10-2473⟩. ⟨hal-02650265⟩
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