Long term outcome of bosentan treatment in primary pulmonary hypertension (PPH) and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases (PAH-SSD)

Abstract

transplantation (LTx). In asthma and LTx BOS (Zheng JHLT 1999:18: 231) increased submucosal vascularity has been shown to contribute to airflow limitation. Vascularity has 2 components: sprouting angiogenesis(more vessels) and microvascular enlargement(larger vessels). We hypothesise that the lack of bronchial arterial reanastomosis at the time of transplant may stimulate angiogenesis and be a risk factor for subsequent BOS. Aim: To determine the components and time course in relation to BOS of post-LTx airway vasculature Methods: 27 initially stable(BOS 0) LTx recipients were recruited at 148 80 days post-LTx and followed over 3 years. 8 remained stable and 19 developed BOS. 9 normal controls were also recruited. Airway vasculature was examined immunohistochemically in endobronchial biopsies(EBB) with mouse antihuman collagenIV antibody, quantified by computer image analyser, expressed as average vessel size m, vessel number/mm of lamina propria and overall %vascularity. Demographic, clinical and EBB findings were statistically analysed. Results: There was significant increased baseline vessel size and vascularity, but not vessel numbers in both stable and BOS LTx compared to normals.