Journal Article

CXCL13 in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Fuge, Jan, Lerch, Christian, Schiffer, Lena, Olsson, Karen M., Welte, Tobias, Olle, Sandra, Hoeper, Marius M., Haller, Hermann, Jonigk, Danny, Maegel, Lavinia

2016

Overview

Background Chemokine CXC ligand 13 (CXCL13) has been implicated in perivascular inflammation and pulmonary vascular remodeling in patients with idiopathic pulmonary artery hypertension (IPAH). We wondered whether CXCL13 may also play a role in chronic thromboembolic pulmonary hypertension (CTEPH) and whether serum levels of CXCL13 might serve as biomarkers in these conditions. Methods Lung tissue from patients with IPAH or CTEPH was immunostained for CXCL13. Serum samples were obtained from patients with IPAH ( n  = 42) or CTEPH ( n  = 50) and from healthy controls ( n  = 13). Serum CXCL13 concentrations were measured by enzyme-linked immunosorbent assay technology and were evaluated for associations with markers of disease severity and survival. Results CXCL13 was expressed in pulmonary vascular lesions and lymphocytes of patients with IPAH and inoperable CTEPH, respectively. Serum CXCL13 was elevated in patients compared to healthy controls [median, interquartile range, 83 (55,114) pg/ml versus 40 (28, 48) pg/ml; p  < 0.001]. Serum CXCL13 showed only weak and inconsistent correlations with markers of inflammation or disease severity. In both populations, patients with serum CXCL13 above the median of the respective groups did not have a higher risk of death than patients with lower serum CXCL13. Conclusions CXCL13 was overexpressed in pulmonary vascular lesions of patients with IPAH and CTEPH, and increased serum concentrations were found in patients with IPAH and CTEPH, suggesting a potential pathogenic role of CXCL13 in both diseases. However, given the weak associations between serum CXCL13 and markers of disease severity and outcome, CXCL13 is unlikely to become a promising biomarker in these patient populations.

• Publisher: BioMed Central,BioMed Central Ltd,Nature Publishing Group
• Subject: Age Distribution / Aged / Analysis / Biomarkers - blood / Care and treatment / Chemokine CXCL13 - blood / Chronic Disease / Complications and side effects / Enzyme-linked immunosorbent assay / Familial Primary Pulmonary Hypertension - blood / Familial Primary Pulmonary Hypertension - diagnosis / Familial Primary Pulmonary Hypertension - mortality / Female / Gene expression / Germany - epidemiology / Humans / Hypertension / Lesions / Lymphocytes / Male / Medicine / Medicine & Public Health / Middle Aged / Pneumology/Respiratory System / Prevalence / Pulmonary Embolism - blood / Pulmonary Embolism - diagnosis / Pulmonary Embolism - mortality / Pulmonary hypertension / Reproducibility of Results / Risk Factors / Sensitivity and Specificity / Sex Distribution / Survival Rate