Details

Autor(en) / Beteiligte

• Guillén-Del-Castillo, Alfredo
• Meseguer, Manuel López
• Fonollosa-Pla, Vicent
• Giménez, Berta Sáez
• Colunga-Argüelles, Dolores
• Revilla-López, Eva
• Rubio-Rivas, Manuel
• Ropero, Maria Jose Cristo
• Argibay, Ana
• Mir, Joan Albert Barberá
• Salas, Xavier Pla
• Meñaca, Amaya Martínez
• Vuelta, Ana Belén Madroñero
• Padrón, Antonio Lara
• Comet, Luis Sáez
• Morera, Juan Antonio Domingo
• González-Echávarri, Cristina
• Mombiela, Teresa
• Ortego-Centeno, Norberto
• González, Manuela Marín
• Tolosa-Vilella, Carles
• Blanco, Isabel
• Subías, Pilar Escribano
• Simeón-Aznar, Carmen Pilar

Titel

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

Ist Teil von

• Scientific reports, 2022-03, Vol.12 (1), p.5289-5289, Article 5289

Ort / Verlag

England: Nature Publishing Group

Erscheinungsjahr

2022

Link zum Volltext

Quelle

Free E-Journal (出版社公開部分のみ）

Beschreibungen/Notizen

• 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.