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Clinical characteristics and survival in systemic sclerosis-related PH associated With ILD.
Launay D, Humbert M, Berezne A, Cottin V, Allanore Y, Couderc LJ, Bletry O, Yaici A, Hatron PY, Mouthon L, Le Pavec J, Clerson P, Hachulla E.
BACKGROUND:
Pulmonary hypertension (PH) complicating systemic sclerosis (SSc)-related interstitial lung disease (ILD) (PH-ILD) is usually associated with a poor prognosis. However, data are either lacking or scarce on prognosis factors in this condition. The objectives of the study were to compare the survival of patients with 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 heart catheterization were included in a cross-sectional analysis. PH was classified as mild (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 males with an history of smoking, had more frequently diffuse SSc, less frequently anticentromere antibodies and a lower FVC/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:
SSc patients 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.