The power of the EUSTAR cohort: key findings to date and implications for management of systemic sclerosis patients

ABSTRACT

Introduction

The rarity of systemic sclerosis (SSc) and its widely heterogeneous presentation and disease course are the main limitations for clinical research. The European Scleroderma Trials and Research group (EUSTAR) was launchegd in 2004, aiming to unify research efforts in the field of SSc. The central EUSTAR database has grown exponentially over the years, promoting new research and clinical trials, shedding new light on SSc diagnosis, its clinical course and providing new ideas for state-of-the-art therapy.Areas covered: The authors summarized the key findings of the main EUSTAR studies by reviewing PubMed and Web-of-Science databases through July 2020. The authors focused on the very early diagnosis of SSc, the prediction of disease course and mortality, the evaluation of disease activity and quality of life, the general management and therapy.

Expert opinion

The findings elucidated in EUSTAR studies have substantially improved the diagnostic and therapeutic approach to SSc in the last 15 years. Further efforts are warranted to identify early prognostic markers of the disease and stratify patients who may benefit most from vasoactive, immunosuppressive, and/or antifibrotic therapy. This will be particularly important in leading the future of SSc toward precision medicine and to promote more targeted clinical trials.

KEYWORDS:

• Systemic sclerosis
• EUSTAR
• general management
• epidemiology
• disease activity
• prognostic factors
• therapy
• recent insights

Declaration of interest

E Zanatta received consultancy fees from GlaxoSmithKline, Actelion and Boehringer Ingelheim.

J Avouac has/had consultancy relationships and/or has received research funding in relation to the treatment of systemic sclerosis from Actelion, Boehringer Ingelheim, Roche, Pfizer, Sanofi, and Bristol-Myers Squibb.

AM Hoffman-Vold received consulting fees and/or research funding from Actelion, ARXX, Bayer, Boehringer Ingelheim, Medscape, MSD and Roche.

O Distler has/had consultancy relationship and/or has received research funding from (last 3 years): Abbvie, Acceleron Pharma, Amgen, AnaMar, Bayer, Boehringer Ingelheim, Catenion, Drug Development International Ltd, CSL Behring, ChemomAb, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, Italfarmaco, iQvia, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Target Bio Science and UCB in the area of potential treatments of scleroderma and its complications. He has a patent mir-29 for the treatment of systemic sclerosis issued (US8247389, EP2331143).

M Matucci-Cerinic received consulting fees, honoraria or research funding from Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, Roche.

Y Allanore had consultancy relationships and/or has received research funding from Actelion, Bayer, Boehringer Ingelheim, ChemomAb, Genentech/Roche, Inventiva, Pfizer, Sanofi, Servier, in the area of potential treatments of scleroderma and its complications.

A Doria received honoraria/speaking fees from GSK, Eli-Lilly, Roche, Janssen, Pfizer.

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. No writing assistance was utilized in the production of this manuscript.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Author contributions

Francesco Benvenuti and Elisabetta Zanatta contributed equally to the article.

Additional information

Funding

This paper was not funded.