PT - JOURNAL ARTICLE AU - Eva Padrão AU - Vanessa Santos AU - Patrícia Caetano Mota AU - Natália Melo AU - Rui Cunha AU - José Miguel Pereira AU - António Morais TI - Usual interstitial pneumonia pattern in chronic hypersensitivity pneumonitis AID - 10.1183/13993003.congress-2016.PA800 DP - 2016 Sep 01 TA - European Respiratory Journal PG - PA800 VI - 48 IP - suppl 60 4099 - http://erj.ersjournals.com/content/48/suppl_60/PA800.short 4100 - http://erj.ersjournals.com/content/48/suppl_60/PA800.full SO - Eur Respir J2016 Sep 01; 48 AB - Introduction: There is a subgroup of patients with chronic hypersensitivity pneumonitis (cHP) and usual interstitial pneumonia pattern (cHP/UIP) which information is scarce.Objective: Characterization of cHP/UIP patients.Methods: Analysis of cHP/UIP patients in whom disease behavior was stratified according with ATS/ERS classification for IIP. HRCT fibrosis score was used for quantification of disease extent.Results: Included 42 patients with mean(SD) age of 65.5±13.3 years, 66.7% female. Fibrosis score average was 8.4±2.8. Regarding disease behavior, 7.1% had stable disease (group-1), 35.7% progressive, irreversible disease with potential for stabilization (group-2) and 35.7% progressive, irreversible disease despite therapy (group-3). Significant differences were found in fibrosis score (group-1vs.3 p=0.038), FVC (group-2vs.3 p=0.02), TLC (group-2vs.3 p=0.006) and DLCO (group-2vs.3 p<0.001). BAL lymphocytosis was present in 100% of group-1, 71.4% of group-2 and 36.4% of group-3 while neutrophilia in 50% of group-1, 78.6% of group-2 and 54.5% of group-3, without significant differences. Therapeutic approach included steroids and immunosuppressants in 63.2%, only steroids in 23.7% and 13.2% didn't make any anti-inflammatory therapy. Eight patients were referred for lung transplantation and there were 10 deaths (23.8%), 7 of which belonged to the group-3.Conclusion: This cohort of patients with cHP/UIP showed a wide variability in disease severity and evolution, however with a significant number of patients having an unfavourable evolution despite therapy. At diagnosis, this subgroup presented a greater functional severity and fibrosis extent and may require higher monitoring and therapeutic approach.