TY - JOUR T1 - Combined pulmonary fibrosis and emphysema: a high-pressure situation JF - European Respiratory Journal JO - Eur Respir J SP - 9 LP - 11 DO - 10.1183/09031936.00156909 VL - 35 IS - 1 AU - J. C. Munson Y1 - 2010/01/01 UR - http://erj.ersjournals.com/content/35/1/9.abstract N2 - The combination of pulmonary fibrosis and emphysema (CPFE) was first described in 1990 by Wiggins et al. 1. Subsequent case studies further described what are now recognised as hallmarks of the syndrome: significant dyspnoea, a predilection for the disease among male smokers, normal or near normal lung volumes resulting from the opposing effects of hyperinflation and fibrosis, and a significantly reduced diffusing capacity 2–4. More recent work has demonstrated that significant pulmonary hypertension is also common in patients with CPFE and associated with shortened survival 5–7. However, these studies relied on transthoracic echocardiography (TTE) without confirmation by right heart catheterisation (RHC) to diagnose pulmonary hypertension, an approach that may have important limitations 8, 9. In this issue of the European Respiratory Journal, Cottin et al. 10 present the results of a retrospective, multicentre cohort study of patients with CPFE and pulmonary hypertension confirmed by RHC. The study cohort was derived from a population of patients meeting diagnostic criteria for CPFE who were reported to a registry of orphan diseases and who subsequently underwent annual TTE to assess for the development of pulmonary hypertension. Patients were referred for RHC by their treating physicians guided by the TTE results. Those with a mean pulmonary artery pressure (P̄pa) >25 mmHg, a pulmonary capillary wedge pressure <15 mmHg and a pulmonary vascular resistance (PVR) >240 dyn·s·cm−5 were included in the analysis. The patient cohort had a median follow-up after RHC of 8 months. This study not only confirms many of the clinical characteristics of CPFE described in prior studies but also … ER -