Enhanced Liver Fibrosis Score as a Biomarker for Vascular Damage Assessment in Patients with Takayasu Arteritis—A Pilot Study

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.

Novel Th17 Lymphocyte Populations, Th17.1 and PD1+Th17, are Increased in Takayasu Arteritis, and Both Th17 and Th17.1 Sub-Populations Associate with Active Disease

“…Recent literature also suggests that circulating markers of fibrosis associate with vascular damage in TAK. 29 It is reasonable to hypothesize that PD1+ CD4+ T lymphocytes and PD1+ Th17 lymphocytes might drive vascular fibrosis in TAK. Other T lymphocyte populations that have been recently implicated in the pathogenesis of TAK include cytotoxic T lymphocytes as well as dysfunctional regulatory T lymphocytes (Treg).…”

Takayasu Arteritis: A Difficult Diagnosis in a Patient With an Extensive Cardiovascular History

“…Liver fibrosis is a dynamic state that reflects an imbalance between metalloproteinases and tissue inhibitors of metalloproteinases, resulting in the deposition of collagen fibers. As one of the main hallmarks of Takayasu arteritis is endothelial damage, it has been hypothesized that this scoring system could accurately quantify vascular damage [ 9 ].…”

“…As our patient had a biopsy that at the time it was done, it didn't entirely exclude the diagnosis of vasculitis, it is important to mention that presently, the biopsy is particularly difficult to perform, as most of the arteries affected are extracranial. In this case, it is advisable to harvest the material in the event of surgery, which was the case when he had a CABG [ 9 ]. One of the findings most commonly associated with large vessel vasculitides is aortitis; however, there are other autoimmune causes, such as Behcet's disease, ankylosing spondylitis, rheumatoid arthritis, lupus, and immunoglobulin G4 (IgG4) related disease that could potentially account for these findings.…”

Arterial wall fibrosis in Takayasu arteritis and its potential for therapeutic modulation

“…Another recent study explored the enhanced liver fibrosis (ELF) score, a validated circulating biomarker of liver fibrosis derived from serum levels of hyaluronic acid (HA), tissue inhibitor of metalloproteinase 1 (TIMP-1), and pro-collagen III amino-terminal pro-peptide (PIIINP), in 24 patients with TAK. The ELF score moderately correlated with clinical damage indices VDI and TADS and strongly correlated with angiographically assessed vascular damage using the Combined Arteritis Damage Score ( 52 ).…”