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
| Autor: | Ignacio Blanco, V. Fonollosa-Pla, Alfredo Guillén-Del-Castillo, A. Marín Ballvé, A. Romás Broto, D. Colunga Argüelles, R Lopez Reyes, A. Castro, C. P. Simeón-Aznar, P Escribano Subias, E. Sala Llinas, I. Otero González, C. Tolosa, Manuel López-Meseguer |
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| Rok vydání: | 2020 |
| Předmět: |
030203 arthritis & rheumatology
0301 basic medicine medicine.medical_specialty Vital capacity business.industry Immunology Interstitial lung disease Retrospective cohort study medicine.disease General Biochemistry Genetics and Molecular Biology Pulmonary function testing 03 medical and health sciences FEV1/FVC ratio 030104 developmental biology 0302 clinical medicine Rheumatology DLCO Internal medicine Diffusing capacity medicine.artery Pulmonary artery medicine Immunology and Allergy business |
| Zdroj: | Annals of the Rheumatic Diseases. 79:705.2-705 |
| ISSN: | 1468-2060 0003-4967 |
| Popis: | 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; PConclusion: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. |
| Databáze: | OpenAIRE |
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