Abstract

Takayasu Arteritis (TA) is characterized by granulomatous panarteritis, vessel wall fibrosis, and irreversible vascular impairment. The aim of this study is to explore the usefulness of the Enhanced Liver Fibrosis score (ELF), procollagen-III aminoterminal propeptide (PIIINP), tissue inhibitor of matrix metalloproteinase-1 (TIMP-1), and hyaluronic acid (HA) in assessing vascular damage in TA patients. ELF, PIIINP, TIMP-1, and HA were measured in 24 TA patients, and the results were correlated with the clinical damage indexes (VDI and TADS), an imaging damage score (CARDS), and disease activity scores (NIH and ITAS2010). A mean ELF score 8.42 (±1.12) and values higher than 7.7 (cut-off for liver fibrosis) in 21/24 (87.5%) of patients were detected. The VDI and TADS correlated significantly to ELF (p < 0.01). Additionally, a strong association across ELF and CARDS (p < 0.0001), PIIINP and CARDS (p < 0.001), and HA and CARDS (p < 0.001) was observed. No correlations of the tested biomarkers with inflammatory parameters, NIH, and ITAS2010 scores were found. To our knowledge, this is the first study that suggests the association of the serum biomarkers PIIINP, HA, and ELF score with damage but not with disease activity in TA patients. The ELF score and PIIINP may be useful biomarkers reflecting an ongoing fibrotic process and quantifying vascular damage.

Highlights

  • It has been demonstrated that the vasculitogenic T cells migration, through the type IV collagen-containing basement membrane, might be enabled by matrix metalloproteinases (MMP)-9, which is abundantly produced by macrophages [5]

  • This study aims to investigate the hypothesis that quantitative values of Enhanced Liver Fibrosis score (ELF) and its constituents, tissue inhibitor of matrix metalloproteinase-1 (TIMP-1), procollagen-III aminoterminal propeptide (PIIINP), and hyaluronic acid (HA), may demonstrate fibrosis of blood vessels and serve as markers for disease extent and damage in Takayasu Arteritis (TA) patients

  • A blood sample for PIIINP, TIMP-1, and HA analysis to perform the ELF calculation was taken from all TA patients who agreed to participate in the study and provided their informed consent

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Summary

Introduction

Takayasu Arteritis (TA) is a rare, idiopathic, chronic inflammatory disease characterized by granulomatous panarteritis of the aorta and its major branches [1]. Accumulating evidence supports the possibility that matrix metalloproteinases (MMP)-2, MMP-3, and MMP-9 may be sensitive biomarkers for TA activity [2]. The transcriptional expression of MMP-1, -3, -9, and the tissue inhibitor of matrix metalloproteinase-1 (TIMP-1) in peripheral. T cells from TA patients has been studied to reveal the influencing factors for activating the adventitial fibroblasts [3]. Activated MMP-2/MMP-9 were demonstrated to stimulate myofibroblasts and increase the influx of monocytes/macrophages, leading to oxidative stress, inflammation, and vascular wall injury [4]. It has been demonstrated that the vasculitogenic T cells migration, through the type IV collagen-containing basement membrane, might be enabled by MMP-9, which is abundantly produced by macrophages [5]

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