The presence of autoantibodies against extractable nuclear antigens (ENAs) in bronchoalveolar lavage (BAL) has been demonstrated in a previous series of 155 patients with interstitial lung disease (ILD). Aim: to analyze the clinical, functional, and radiological differences of the subgroup of patients diagnosed with Interstitial Pneumonia with Autoimmune Features (IPAF) and connective tissue disease-related ILD (CTD-ILD) who presented positivity for ENAs in the BAL (ENAs+) compared with those negative at 3 years of follow-up (ENAs -). Methods: A double-center prospective, longitudinal, observational study was performed. Results: 48 patients (32 women, median age 65 yrs) were included; ENAs+(n)19 and ENAs- 29. Radiological progression of ILD was similar in both groups 21% (4/19) of the ENAs+ group and 20% (6/29) of the ENAS - group,(p=NS).14 of the 48 patients died during the follow-up period: ENAs+ n(%) 7(36), ENAs - 7(24), p=0.227.The most common causes of death were exacerbation and/or progression of ILD in 6 cases (43%), cancer in 5 (36%), and infectious (bacterial or viral) in 3 (21%). Conclusions: Despite the increased mortality in the ENAs+ group, the positivity of ENAs in the BAL was not associated with any of the variables studied. It would be necessary to increase the sample of patients to define the prognostic value of autoantibodies in BAL.