Evidence-based management of rapidly progressing systemic sclerosis

Abstract

Systemic sclerosis has the highest case-specific mortality of any of the auto-immune rheumatic diseases, as well as causing major morbidity. It is a major clinical challenge and one that has previously provoked substantial nihilism due to the limited therapeutic options available and the perceived lack of evidence for clinical effectiveness of those treatments that are currently in use. However, this situation is changing; there are emerging data supporting efficacy for some treatment approaches for this patient group together with a growing number of exciting potential novel approaches to treatment that are moving into the clinical arena. Some of the recent clinical trials are reviewed and discussed in detail.

FIG A. Diagnostic approach in patients with systemic sclerosis

•Subset classification important as severe progressive skin disease associates with increased risk of renal crisis and cardiac involvement •Disease duration reflects risk of major internal organ disease. Renal crisis in early dcSSc and severe lung fibrosis more likely in severe SSc of early onset; both less likely after 5 years. Gastrointestinal involvement, cardiac disease and PAH can occur in early and later disease. *Disease duration is variable in an individual pt and continue monitoring till stabilization/improvement of skin dz

FIG B. Algorithm for treatment of severe progressive systemic sclerosis

*Selective serotonin reuptake inhibitors