FRI0242 IMPACT OF PULMONARY ARTERIAL HYPERTENSION WITH OR WITHOUT INTERSTITIAL LUNG DISEASE ON SCLERODERMA: A RETROSPECTIVE COHORT STUDY FROM THE NATIONWIDE SPANISH SCLERODERMA (RESCLE) AND PULMONARY ARTERIAL HYPERTENSION (REHAP) REGISTRIES

Abstract

Background:Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are the major life-threatening complications in systemic sclerosis (SSc). Data on the impact of PAH and/or ILD in SSc patients (pts) are limited by their low prevalence.Objectives:To assess differences in demographic/clinical characteristics of SSc pts according to presence of PAH and how these are affected by ILD. The impact on characteristics and survival of PAH + ILD was also assessed.Methods:We compared data on SSc pts without PAH from the Spanish registry of patients with SSc (RESCLE) (SSc pts) and SSc pts with PAH from the Spanish registry of pts with PAH (REHAP) (SSc-PAH pts). Only data common in both registries were analyzed. Sub analyses were performed according to the presence/absence of ILD. Transplant-free survival from diagnosis of PAH was estimated using the Kaplan-Meier method.Results:1,579 pts with SSc (RESCLE) and 364 pts with SSc-PAH (REHAP) were analyzed. Compared to SSc pts, SSc-PAH pts had worse functional status (NYHA FC III/IV: 70.6% vs. 8.2% in SSc pts) and pulmonary function (lower mean forced vital capacity [FVC, 81.2±20.6% vs. 93.6±20] and diffusing capacity for carbon monoxide [DLCO, 45.3±17.7% vs. 79.0±36.6%]). More patients had FVC/DLCO ≥1.4 (77.8% vs. 34.8%), tricuspid regurgitation (91.4% vs. 46.1%) or pericardial effusion (30.0% vs. 5.1%). Mean systolic pulmonary artery pressure (sPAP) was higher (70.0±21.3 vs. 27.5±9.1 mmHg) (all respectively; P<0.001 for all). Prevalence of ILD on high-resolution computerized tomography was similar (44.9% [n=92] vs. 41.8% [n=422] in SSc pts; P=0.408). These differences were also found when splitting both cohorts according to the presence / absence of ILD. Compared to SSc-PAH pts without ILD (n=128), pts with PAH + ILD (n=92) had worse functional status (NYHA FC III: 69.6% vs. 55.5% in SSc-PAH pts without ILD; P=0.036), lower mean FVC (70.9±21.9 vs. 86.2±18.6) and lower DLCO (39.4 ±17.0 vs. 49.1±17.9) both P<0.001. Five-year survival rate was 35% in SSc-PAH pts with ILD vs. 45% in SSc-PAH without ILD (P=0.444 [figure 1]).Conclusion:PAH has a profound impact on functional status, pulmonary function and right ventricle function of SSc patients, independently of presence of ILD. Despite the deleterious effect of functional status and pulmonary function, in pts with SSc and PAH, presence of concomitant ILD has no impact on 5-year survival.Disclosure of Interests:Alfredo Guillén-del-Castillo Consultant of: Actelion pharmaceuticals, GSK, MSD., Manuel López-Meseguer Consultant of: Actelion pharmaceuticals, GSK, MSD., Carles Tolosa Consultant of: Actelion pharmaceuticals, GSK, MSD., Antonio Romás Broto Consultant of: Actelion pharmaceuticals, GSK, MSD., Dolores Colunga Argüelles Consultant of: Actelion pharmaceuticals, GSK, MSD., Isabel Blanco Consultant of: Actelion pharmaceuticals, GSK, MSD., Antoni Castro Consultant of: Actelion pharmaceuticals, GSK, MSD., Raquel López Reyes Consultant of: Actelion pharmaceuticals, GSK, MSD., Adela Marín Ballvé: None declared, Isabel Otero González Consultant of: Actelion pharmaceuticals, GSK, MSD., Vicent Fonollosa-Pla Consultant of: Actelion pharmaceuticals, GSK, MSD., Ernest Sala LLinas Consultant of: Actelion pharmaceuticals, GSK, MSD., Pilar Escribano Subías Consultant of: Actelion pharmaceuticals, GSK, MSD., Carmen Pilar Simeón-Aznar Consultant of: Actelion pharmaceuticals, GSK, MSD.