Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.

Autor: Guillén-Del-Castillo A; Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain., Meseguer ML; Pneumology Department, Hospital Universitario Vall d'Hebrón, Passeig Vall d'Hebron 119-129, 08035, Barcelona, Spain. manuelop@vhebron.net., Fonollosa-Pla V; Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain., Giménez BS; Pneumology Department, Hospital Universitario Vall d'Hebrón, Passeig Vall d'Hebron 119-129, 08035, Barcelona, Spain.; Physiology Department, Universitat Autònoma de Barcelona, Barcelona, Spain., Colunga-Argüelles D; Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain., Revilla-López E; Pneumology Department, Hospital Universitario Vall d'Hebrón, Passeig Vall d'Hebron 119-129, 08035, Barcelona, Spain., Rubio-Rivas M; Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario de Bellvitge-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain., Ropero MJC; Pulmonary Hypertension Unit, Cardiology Department of Hospital, Universitario, 12 de Octubre, Madrid, Spain., Argibay A; Unit of Systemic Autoimmune Diseases and Thrombosis, Department of Internal Medicine, Complejo Hospitalario Universitario de Vigo, Vigo, Pontevedra, Spain., Barberá JA; Pulmonary Medicine Department, Hospital Clínic de Barcelona/Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Madrid, Spain., Salas XP; Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Consorci Hospitalari de Vic, Vic, Barcelona, Spain., Meñaca AM; Pneumology Department, Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain., Vuelta ABM; Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain., Padrón AL; Cardiology Department, Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain., Comet LS; Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain., Morera JAD; Pneumology Department, Hospital Universitario Miguel Servet, Zaragoza, Spain., González-Echávarri C; Autoimmune Diseases Research Unit, Department of Internal Medicine, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, University of the Basque Country, Barakaldo, Spain., Mombiela T; Cardiology Department, Hospital Universitario Gregorio Marañón, Madrid, Spain., Ortego-Centeno N; Inst Invest Biosanitaria Ibs Granada, Department of Internal Medicine, Unit of Systemic Autoimmune Diseases, Hospital Universitario San Cecilio, Granada, Spain.; Department of Medicine, Facultad de Medicina, Hospital Universitario San Cecilio, Granada, Spain., González MM; Pneumology Department, Hospital Clínico Universitario de Valencia, Valencia, Spain., Tolosa-Vilella C; Department of Internal Medicine, Parc Taulí, Hospital Universitario, Sabadell, Barcelona, Spain., Blanco I; Pulmonary Medicine Department, Hospital Clínic de Barcelona/Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Madrid, Spain., Subías PE; Pulmonary Hypertension Unit, Cardiology Department of Hospital, Universitario, 12 de Octubre, Madrid, Spain.; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV)/Instituto de Salud Carlos III, Madrid, Spain., Simeón-Aznar CP; Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
Jazyk: angličtina
Zdroj: Scientific reports [Sci Rep] 2022 Mar 28; Vol. 12 (1), pp. 5289. Date of Electronic Publication: 2022 Mar 28.
DOI: 10.1038/s41598-022-09353-z
Abstrakt: To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P < 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 ± 5.2 mm vs 19.9 ± 6.7 mm, P < 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P < 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P < 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment.
(© 2022. The Author(s).)
Databáze: MEDLINE
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