Source: International Congress 2014 – ILDs 6
Disease area: Interstitial lung diseases, Pulmonary vascular diseases

Abstract

IntroductionCombined idiopathic pulmonary fibrosis with emphysema (CPFE) is characterized by upper lobe emphysema and lower lobe fibrosis, and has got special attention, especially because of the higher association with lung cancer and pulmonary hypertension.MethodsRegistration of patients with CPFE from 10/2009 to 9/2013 in Hemer Lung Clinic. Review of lung functions, data of cardiopulmonary exercise testing and occurrence of lung cancer.ResultsFrom 11/2009 to 10/2013 we identified 20 patients (4 [female] / 16 [male]) with CPFE. Mean age was 64.9 years, BMI 26.6 kg/m², FEV1 83.7 %-pred and VC 94.13%-pred, RV 109.5%-pred, TLC 98.2%-pred and RV%/TLC 102.7 %-pred. R_tot and SR_tot were in normal range. TLCO was reduced to 36%-pred and TLCO/VA to 48.6%-pred. 3 patients had lung cancer. 5 patients had pumonary hypertension (PH). 3 patients with and 6 without PH were available for cardiopulmonary exercise testing. AaDO₂ in CPFE+PH compared to CPFE was higher at VT1 (58 mmHg vs. 48.1 mmHg) and peak exercise (69.3 mmHg vs. 54.5 mmHg). Maximum exercise levels were 65 vs. 82 watt. Peak-VO₂ was lower in CPFE+PH (933 ml vs. 1264 ml) with a trend to lower VO₂/min/watt in COPD+PH. Dead space ventilation (57.9% vs. 57.5%) and end-tidal CO₂ (24.5 vs 24.9 mmHg) were not significantly different between both groups.DiscussionOur results are consistent with previously reported lung function and anthropometic data of CPFE. There is a higher prevalence of PH and lung cancer compared to patients with COPD or IPF. CPX-testing shows severe functional impairment and may helpful in identifying patients with concomitant PH and circulatory impairment.

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M. Westhoff, P. Litterst (Hemer, Germany). Clinical data, lung function and cardiopulmonary exercise testing in CPFE (combined pulmonary fibrosis with emphysema). Results of a cohort of 20 patients. Eur Respir J 2014; 44: Suppl. 58, 3796

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