Acute exacerbation in combined pulmonary fibrosis and emphysema (CPFE): what special features?

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.