Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension.
Por:
Guillen-Del-Castillo A, Meseguer M, Fonollosa-Pla V, Gimenez B, Colunga-Arguelles D, Revilla-Lopez E, Rubio-Rivas M, Ropero M, Argibay A, Mir J, Salas X, Menaca A, Vuelta A, Padron A, Comet L, Morera J, Gonzalez-Echavarri C, Mombiela T, Ortego-Centeno N, Gonzalez M, Tolosa-Vilella C, Blanco I, Subias P, Simeon-Aznar C, RESCLE Consortium, REHAP Consortium
Publicada:
28 mar 2022
Ahead of Print:
28 mar 2022
Resumen:
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.
Filiaciones:
Guillen-Del-Castillo A:
Hosp Univ Vall dHebron, Dept Internal Med, Unit Autoimmune Dis, Barcelona, Spain
Meseguer M:
Hosp Univ Vall dHebron, Pneumol Dept, Passeig Vall dHebron 119-129, Barcelona 08035, Spain
Fonollosa-Pla V:
Hosp Univ Vall dHebron, Dept Internal Med, Unit Autoimmune Dis, Barcelona, Spain
Gimenez B:
Hosp Univ Vall dHebron, Pneumol Dept, Passeig Vall dHebron 119-129, Barcelona 08035, Spain
Univ Autonoma Barcelona, Physiol Dept, Barcelona, Spain
Colunga-Arguelles D:
Hosp Univ Cent Asturias, Dept Internal Med, Oviedo, Asturias, Spain
Revilla-Lopez E:
Hosp Univ Vall dHebron, Pneumol Dept, Passeig Vall dHebron 119-129, Barcelona 08035, Spain
Rubio-Rivas M:
Hosp Univ Bellvitge IDIBELL, Unit Autoimmune Dis, Dept Internal Med, Barcelona, Spain
Ropero M:
Univ 12 Octubre, Cardiol Dept Hosp, Pulm Hypertens Unit, Madrid, Spain
Argibay A:
Complejo Hosp Univ Vigo, Dept Internal Med, Unit Syst Autoimmune Dis & Thrombosis, Vigo, Pontevedra, Spain
Mir J:
Hosp Clin Barcelona, Pulm Med Dept, Inst Invest Biomed August Pi & Sunyer IDIBAPS, Barcelona, Spain
Ctr Invest Biomed Red Enfermedades Resp IDIBAPS, Madrid, Spain
Salas X:
Consorci Hosp Vic, Unit Syst Autoimmune Dis, Dept Internal Med, Barcelona, Spain
Menaca A:
Hosp Univ Marques de Valdecilla, Pneumol Dept, Santander, Cantabria, Spain
Vuelta A:
Hosp Gen San Jorge, Dept Internal Med, Huesca, Spain
Padron A:
Hosp Univ Canarias, Cardiol Dept, Santa Cruz De Tenerife, Spain
Comet L:
Hosp Univ Miguel Servet, Dept Internal Med, Zaragoza, Spain
Morera J:
Hosp Univ Miguel Servet, Pneumol Dept, Zaragoza, Spain
Gonzalez-Echavarri C:
Univ Basque Country, Autoimmune Dis Res Unit, Dept Internal Med, Biocruces Bizkaia Hlth Res Inst, Baracaldo, Spain
Mombiela T:
Hosp Univ Gregorio Maranon, Cardiol Dept, Madrid, Spain
Ortego-Centeno N:
Hosp Univ San Cecilio, Inst Invest Biosanitaria Ibs Granada, Dept Internal Med, Unit Syst Autoimmune Dis, Granada, Spain
Hosp Univ San Cecilio, Fac Med, Dept Med, Granada, Spain
Gonzalez M:
Hosp Clin Univ Valencia, Pneumol Dept, Valencia, Spain
Tolosa-Vilella C:
Hosp Univ, Dept Internal Med, Parc Tauli, Barcelona, Spain
Blanco I:
Hosp Clin Barcelona, Pulm Med Dept, Inst Invest Biomed August Pi & Sunyer IDIBAPS, Barcelona, Spain
Ctr Invest Biomed Red Enfermedades Resp IDIBAPS, Madrid, Spain
Subias P:
Univ 12 Octubre, Cardiol Dept Hosp, Pulm Hypertens Unit, Madrid, Spain
Inst Salud Carlos III, Ctr Invest Biomed Red Enfermedades Cardiovasc CIB, Madrid, Spain
Simeon-Aznar C:
Hosp Univ Vall dHebron, Dept Internal Med, Unit Autoimmune Dis, Barcelona, Spain
RESCLE Consortium:
RESCLE Consortium
REHAP Consortium:
REHAP Consortium
gold, Green Published
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