Guidelines and Recommendations Towards Evidence-Based Management of Systemic Sclerosis

Abstract

The preferred treatment of systemic sclerosis (SSc) is unclear due to an incomplete understanding of disease pathogenesis, variations in disease manifestations and variable quality of evidence to support treatment decisions. The objectives of this article are to discuss the value of clinical practice guidelines, critique guideline development and uptake, compare SSc-specific guidelines and highlight consensus-based treatment algorithms to complement guidelines when recommendations and/or high-quality evidence are lacking. Thirty-nine guidelines or recommendations for the screening, diagnosis, treatment and conduct of trials in SSc have been published. Four publications contained guidance on the treatment of multiple domains. The European League Against Rheumatism and the EULAR Scleroderma Trial and Research group guidelines were published in 2009 and updated in 2017. The British Society of Rheumatology and the British Health Professionals in Rheumatology published guidelines in 2016. The Brazilian Society of Rheumatology published SSc guidelines in 2013. The absence of recommendations for the preferred order of therapy is a limitation of all published guidelines. Treatment algorithms, based on SSc expert consensus, were proposed in 2012 and updated in 2018. These algorithms are complementary to the guidelines, as they provide expert consensus in areas of uncertainty. SSc-specific guidelines and treatment algorithms provide evidence-based and/or expert consensus-based recommendations on how to treat patients with the intention of reducing inappropriate practice variability and improving patient outcomes.