Systemic sclerosis is the autoimmune connective tissue disease with the highest morbidity and mortality, through the combination of inflammation, vasculopathy and fibrosis leading to severe internal organ involvement. Currently, there are no approved disease-modifying therapies, and treatment is based on organ-specific treatment and broad immunosuppression, with disappointing long-term results in most cases. Recent research has helped to improve knowledge of the pathogenesis of systemic sclerosis and to optimize treatment based on specific physiopathological targets, and a new era of biological agents in systemic sclerosis has now begun. Promising results are emerging from targeting specific cytokine signalling, especially IL-6, and cellular subpopulations such as B cells, with anti-CD20 therapy, and T-cells, with inhibition of T-cell co-stimulation. Other approaches under evaluation are based on the modulation of profibrotic pathways by anti-TGF-β agents. In this chapter, we discuss the available evidence to support the use of each biological agent in systemic sclerosis based on data from basic and translational research and on results from clinical studies.
Part of the book: Systemic Sclerosis