Combined pulmonary fibrosis-emphysema syndrome

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Combined pulmonary fibrosis-emphysema syndrome

Synonyms: CPFE

A rare interstitial lung disease characterized by the coexistence of emphysema and usual interstitial pneumonia typically occurring in male smokers. Emphysema is usually encountered in the upper lobes preceding fibrosis of the lower lobes. Patients present with severe dyspnea and markedly reduced diffusion capacity on functional testing while spirometric values are relatively preserved. The syndrome is frequently complicated by pulmonary hypertension and acute lung injury.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: // Data version September 2023.

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Advocacy Organizations

Breathe Support Network

MISSION STATEMENT The Breathe Support Networks mission is to provide support and education for pulmonary fibrosis patients and their families, helping them to better #LIVEwithPF. This includes, but is not limited to: - education about pulmonary fibrosis - guiding patients and families on how to talk to their healthcare team - providing tips to live a healthier more productive life - providing information about other pulmonary fibrosis resources

Clinical Trials

For a list of clinical trials in this disease area, please click here.