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This review discusses combined pulmonary fibrosis and emphysema (CPFE) in the setting of connective tissue disease.CPFE is a recently identified syndrome in smokers or ex-smokers characterized by dyspnea often severe, preserved lung volumes, severely impaired gas exchanges, and an increased risk of pulmonary hypertension associated with a dismal prognosis, and possibly lung cancer. It may be encountered in the setting of connective tissue diseases, especially rheumatoid arthritis and systemic sclerosis, with generally similar features as ‘idiopathic’ (tobacco-related) CPFE. The diagnosis is based on the presence of both emphysema predominating in the upper lobes and frequently paraseptal, and interstitial abnormalities suggesting pulmonary fibrosis in the lower lung zones with velcro crackles at auscultation. Pathologic radiological correlations are difficult owing to various pathology and difficulties in identifying honeycombing at chest high-resolution computed tomography in the setting of coexistent emphysema. Tobacco smoking is associated with an increased risk of developing most of the individual components of the syndrome (i.e. emphysema, pulmonary fibrosis, pulmonary hypertension, rheumatoid arthritis, and pulmonary fibrosis among patients with rheumatoid arthritis). CPFE impacts modalities of follow-up for pulmonary function and detection of pulmonary hypertension especially in systemic sclerosis.The syndrome of CPFE is a distinct pulmonary manifestation in the spectrum of lung diseases associated with connective tissue diseases, especially in smokers or ex-smokers.