RT Journal Article SR Electronic T1 Impairment of membranous and vascular components of pulmonary diffusion and plasma endothelin-1 in patients with liver cirrhosis with and without COPD JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP p3570 VO 38 IS Suppl 55 A1 Susann Langwieler A1 Thomas Wex A1 Malfertheiner Peter A1 Jens Schreiber YR 2011 UL http://erj.ersjournals.com/content/38/Suppl_55/p3570.abstract AB Liver cirrhosis (LC) may be rarely complicated by hepatopulmonary syndrome or hypertension. Nevertheless abnormalities of gas exchange are frequent in LC. The mechanisms are still unclear. A reduced hepatic clearance of Endothelin – 1 (ET-1) might play a role.In 72 pts. with LC, 29 (40.3%) of whom had accompanying COPD, PFT were performed as well as measurements of PAaDO2, diffusion capacity, pulmonary capillary blood volume (Qc), membrane diffusing capacity (Dm) and plasma ET-1. The functional measurements were performed in a matched group of pts. with COPD without liver function impairment (n=38).None of the pts. had clinically manifest hepatopulmonary syndrome or hypertension. Nevertheless 59/72 (81.9%) of the pts. with LC and all pts. with both LC and COPD and 31/39 (79,5%) pts. with sole COPD showed decreased TLCO. In all pts. but one with sole LC Dm was reduced. Qc was reduced to a lesser extend in 47 (65.2%) pts., with a greater impairment of Dm. In the COPD group the reduction of Dm was the overwhelming mechanism of an abnormal diffusion capacity. PAaDO2 was significantly negatively correlated with TLCO, Qc and to a lesser extend with Dm in pts. with LC without ventilatory impairment. All pts. with LC independently on coexisting COPD showed increased plasma concentratons of ET-1, which were negatively correlated with Qc (r=-0.57, p=0.015).Impairment of the Dm as well as decreased Qc are responsible for an abnormal gas exchange in pts. with LC contrary to COPD where reduction of Dm plays the most important role. Increased ET-1 in LC might contribute to pathogenesis of gas exchange impairment in LC.