Mena-Vázquez, NataliaRojas-Gimenez, MartaRomero-Barco, Carmen MaríaManrique-Arija, SaraHidalgo Conde, AnaArnedo Díez de Los Ríos, RocíoCabrera César, EvaOrtega-Castro, RafaelaEspildora, FranciscoAguilar-Hurtado, María CarmenAñón-Oñate, IsabelPérez-Albaladejo, LorenaAbarca-Costalago, ManuelUreña-Garnica, InmaculadaVelloso-Feijoo, Maria LuisaRedondo-Rodriguez, RocioFernández-Nebro, Antonio2025-01-072025-01-072021-09-282075-4418https://hdl.handle.net/10668/25698To describe the characteristics and progression of interstitial lung disease in patients with associated systemic autoimmune disease (ILD-SAI) and to identify factors associated with progression and mortality. We performed a multicenter, retrospective, observational study of patients with ILD-SAI followed between 2015 and 2020. We collected clinical data and performed pulmonary function testing and high-resolution computed tomography at diagnosis and at the final visit. The main outcome measure at the end of follow-up was forced vital capacity (FVC) >10% or diffusing capacity of the lungs for carbon monoxide >15% and radiological progression or death. Cox regression analysis was performed to identify factors associated with worsening of ILD. We included 204 patients with ILD-SAI: 123 (60.3%) had rheumatoid arthritis (RA), 58 had (28.4%) systemic sclerosis, and 23 (11.3%) had inflammatory myopathy. After a median (IQR) period of 56 (29.8-93.3) months, lung disease had stabilized in 98 patients (48%), improved in 33 (16.1%), and worsened in 44 (21.5%). A total of 29 patients (14.2%) died. Progression and hospitalization were more frequent in patients with RA (p = 0.010). The multivariate analysis showed the independent predictors for worsening of ILD-SAI to be RA (HR, 1.9 [95% CI, 1.3-2.7]), usual interstitial pneumonia pattern (HR, 1.7 [95% CI, 1.0-2.9]), FVC (%) (HR, 2.3 [95% CI, 1.4-3.9]), and smoking (HR, 2.7 [95%CI, 1.6-4.7]). Disease stabilizes or improves after a median of 5 years in more than half of patients with ILD-SAI, although more than one-third die. Data on subgroups and risk factors could help us to predict poorer outcomes.enAttribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/interstitial lung diseaseprognosisrheumatoid arthritissystemic autoimmune diseaseresearch article34679492open access10.3390/diagnostics11101794PMC8534825https://www.mdpi.com/2075-4418/11/10/1794/pdf?version=1632982892https://pmc.ncbi.nlm.nih.gov/articles/PMC8534825/pdf