TY - JOUR T1 - Acute exacerbation in combined pulmonary fibrosis and emphysema (CPFE): what special features? JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2020.5256 VL - 56 IS - suppl 64 SP - 5256 AU - Hana Mrassi AU - Imen Bachouch AU - Nidhal Belloumi AU - Fatma Chermiti AU - Soraya Fenniche Y1 - 2020/09/07 UR - http://erj.ersjournals.com/content/56/suppl_64/5256.abstract N2 - Introduction: The association of emphysema and pulmonary fibrosis was first described as a syndrome in 2005, named CPFE. It is still a rarely recognized clinical entity.Aim: Assess characteristics of acute exacerbations (AE) in CPFE.Methods: Retrospective three-year study of patient records followed for CPFE and having presented AE during follow-up.Results: Sixteen records were reviewed. All patients were men. Mean age was 67.67 ± 9 years. They were all smokers; mean pack-year was 46. Cardiovascular comorbidities were present in 38% of cases. All patients had an exertional dyspnea evolving for 32 months before diagnosis. Fifty percent of them had a mMRC ≥ 3.On average, 1± 0,8 AE happened over the year causing acute respiratory failure and requiring admission. Non-invasive ventilation was needed in 25% of cases.AE were associated with infection in half of cases: sputum culture having identified a germ in 62% of them [Haemophilus influenzae (32%), klebsiella pneumoniae (15%) and Pseudomonas aeruginosa (15%)]. Fatal proximal pulmonary embolism was noted in 12% of cases. However, there was no found cause of AE in 38% of cases.Conclusion:AE in CPFE share characteristics with those in COPD and idiopathic pulmonary fibrosis. No individualized management is yet available.FootnotesCite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 5256.This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -