Combined Pulmonary Fibrosis and Emphysema Syndrome in a Patient Age 28 Years with Severe Systemic Sclerosis: Figure 1.

Abstract

To the Editor: Combined pulmonary fibrosis and emphysema (CPFE) has recently been defined as a distinct syndrome occurring in adult heavy smokers in their sixties and characterized by dyspnea, crackles at lung auscultation, and diagnostic imaging features on high resolution computed tomography (HRCT) of the chest1. Spirometry is often normal or subnormal due to the opposite compensatory effects of pulmonary fibrosis (usually resulting in restrictive lung disease) and of emphysema (usually resulting in obstructive lung disease)1. CPFE has been reported in a series of patients with connective tissue disease (CTD) as a novel hitherto unrecognized pulmonary manifestation2. We describe a “radical” case of CPFE in a young woman with severe systemic sclerosis (SSc) yet with no history of heavy smoking. A 28-year-old woman, an ex-smoker of tobacco (less than 5 pack-years over 10 years) and ex-smoker of cannabis (1 joint per week for 5 years), presented with mild dyspnea. She had no significant environmental or occupational exposure. Limited cutaneous … Address correspondence to Dr. Cottin; E-mail: vincent.cottin{at}chu-lyon.fr