New Approaches in Management of Generalized Scleroderma (Progressive Systemic Sclerosis)

Abstract

Objective of the Review: To demonstrate new approaches in management of generalized scleroderma (GSD), that gained relevance after publication of drug therapy recommendations in 2017. Key Points. GSD is a progressive immunoinflammatory rheumatic disease, that can cause severe damage of vitals; therefore, it is characterized with poor prognosis. Unfortunately, currently there are no approved drugs to modify the course of GSD. Biological disease-modifying agents are still rarely used in GSD, since none of them have approved indications. The recommendations of the European League Against Rheumatism on GSD drug therapy were last updated about 5 years ago. Scientists have been searching for efficient therapies and undertook clinical trials of various medicinal products. The review presents literature data evidencing expanded range of methods to correct immunoregulatory pathologies; discusses results of latest clinical trials, which can provide the basis for inclusion of such drugs into recommendations on GSD management. Conclusion. New randomised controlled trials and multicentre programs point to some progress in GSD curation and better prognosis. Keywords: generalized scleroderma, autologous haematopoietic stem cell transplantation, Rituximab, Tocilizumab, Abatacept, Nintedanib.