Pulmonary vasculopathy in explanted lungs from patients with interstitial lung disease undergoing lung transplantation

Abstract

BackgroundPulmonary hypertension (PH) causes increased morbidity and mortality in patients with interstitial lung diseases (ILD). Classification schemes, while well-characterised for the vasculopathy of idiopathic PH, have been applied, unchallenged, to ILD-related PH. We evaluated pulmonary arterial histopathology in explanted human lung tissue from patients who were transplanted for advanced fibrotic ILD.MethodsLung explants from 38 adult patients who underwent lung transplantation were included. Patients were divided into three groups: none, mild/moderate and severe PH by mean pulmonary artery pressure (mPAP) measured at pre lung transplantation right heart catheterisation (RHC). Grading of pulmonary vasculopathy according to Heath and Edwards scheme, and prelung transplantation evaluation data were compared between the groups.Results38 patients with fibrotic ILDs were included, the majority (21) with idiopathic pulmonary fibrosis. Of the 38 patients, 18 had severe PH, 13 had mild/moderate PH and 7 had no PH by RHC. 16 of 38 patients had severe pulmonary arterial vasculopathy including vascular occlusion with intimal fibrosis and/or plexiform lesions. There were no correlations between mPAP and lung diffusion with the severity of pulmonary arterial pathological grade (Spearman’s rho=0.14, p=0.34, rho=0.11, p=0.49, respectively).ConclusionsPatients with end stage ILD had severe pulmonary arterial vasculopathy in their explanted lungs irrespective of the presence and/or severity of PH as measured by RHC. These findings suggest that advanced pulmonary arterial vasculopathy is common in patients with advanced fibrotic ILD and may develop prior to the clinical detection of PH by RHC.

Combined Pulmonary Fibrosis and Emphysema (CPFE) Clinical Features and Management

“… 2 It was concluded that the presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis. This is in accordance with the findings of Mejía et al 26 As mentioned before, the worse prognosis of CPFE is predominantly caused by the pulmonary hypertension. 25 However, this is in contrast to the statement of Sakai, who believes that the prognosis of CPFE is better than that of IPF/UIP, despite a high prevalence of lung cancer reported in cases with CPFE.…”

“…In a study of 38 lung transplant recipients, who underwent a lung transplantation due to fibrotic end-stage lung disease, there were 21 patients with IPF and 8 with CPFE. 26 In this study, they found significant pulmonary arterial vasculopathy which did not correlate with the clinical severity of the underlying lung disease and did not correlate with the presence and/or severity of PH as measured by right-heart catheterization. 26 In 21%, there were plexiform lesions, which are traditionally a hallmark of WHO group 1 PH, termed PAH.…”

“… 26 In this study, they found significant pulmonary arterial vasculopathy which did not correlate with the clinical severity of the underlying lung disease and did not correlate with the presence and/or severity of PH as measured by right-heart catheterization. 26 In 21%, there were plexiform lesions, which are traditionally a hallmark of WHO group 1 PH, termed PAH. 26 The investigators suggested that the observed vasculopathy might be derived from severe and prolonged hypoxia sharing biochemical mechanisms similar to those seen with WHO group 1 PAH.…”

“… 26 In 21%, there were plexiform lesions, which are traditionally a hallmark of WHO group 1 PH, termed PAH. 26 The investigators suggested that the observed vasculopathy might be derived from severe and prolonged hypoxia sharing biochemical mechanisms similar to those seen with WHO group 1 PAH. They concluded that these findings indicate that advanced pulmonary arteriopathy is common and may develop in a heterogeneous regional pattern in advanced lung disease prior to the clinical detection of PH.…”

“…They concluded that these findings indicate that advanced pulmonary arteriopathy is common and may develop in a heterogeneous regional pattern in advanced lung disease prior to the clinical detection of PH. 26 …”