Enhanced Liver Fibrosis Test: A new biomarker of fibrosis in systemic sclerosis

A multicentre study spread across Europe was recently conducted to evaluate how effective the Enhanced Liver Fibrosis (ELF) Test would be as a biomarker of fibrosis (scarring) in systemic sclerosis (SSc). A biomarker of fibrosis has been an unmet need for a long time and would fulfil the need for a molecular classification of patients with SSc. This in turn would aid the understanding of what drives fibrosis in individuals and thus enable the application of precision medicine for treatment strategies.

It was recently established that the ELF test was a viable test for chronic liver fibrosis, which has previously been shown to be a marker of overall fibrosis in SSc, mainly representing skin and lung involvement. The ELF test is based on an algorithm that comprises the serum concentration of three biological molecules: amino-terminal propeptide of procollagen type III (PIIINP), tissue inhibitor of matrix metalloproteins-1 (TIMP-1), and hyaluronic acid (HA). These are all markers that are involved in fibrosis and collagen remodelling. The principal aim of this study was to hence determine the value of the ELF score and these factors in a multicentre cohort of SSc patients.

The data was collected by acquiring samples from patients at six specialist rheumatic centres in Europe. The samples were analysed to identify the presence of PIIINP, TIMP-1 and HA, alongside statistical analyses. The results from the study indicated that the ELF score and its components are markers of fibrosis in SSc patients and are independently associated with skin and lung involvement. ELF scores were much higher in patients with diffuse cutaneous SSc, severe skin involvement, fibrosis on chest scans and abnormal pulmonary function. There was also significant correlation of HA and of the ELF score with pulmonary hypertension.

As this score is based on a test originally developed for chronic liver fibrosis diseases, a new SSc-specific scoring system is required based on the significance of PIIINP, TIMP-1 and HA as biomarkers in this condition, before the ELF test can be used as a reliable overall biomarker of fibrosis. This is an essential aspect that further studies are focussing on, as by assessing the sensitivity of the 3 components, this may allow predictions to be made for the progression of skin and lung fibrosis in those with SSc. The study may eventually lead to a biomarker that can be used as both a diagnostic tool and a therapeutic target, with the fundamental goals of recognising fibrosis sooner in patients and improving quality of life.

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Information on another piece of new research can be found here: SRUK funding a study to treat fibrosis