What comes before scleroderma?

Abstract

While the classification criteria for systemic sclerosis (SSc) have been carefully delineated, the definition of what comes before meeting classification criteria is not so well understood. In some ways, it is similar to “pre-rheumatoid arthritis” where a reasonable definition has been developed and the downstream early treatment of “pre-rheumatoid arthritis” is being tested. However, for SSc, there may well be a very early SSc phase before any, but constitutional symptoms occur. This preclinical phase is very poorly understood or described. The very early diagnosis of systemic sclerosis (VEDOSS) has been defined, but there remain multiple questions surrounding VEDOSS, including when and how to treat patients with this diagnosis. Despite progress, there are no fully validated biomarkers or genetic predictors for disease evolution. Moreover, although VEDOSS patients with Raynaud’s phenomenon (RP), autoantibodies and SSc capillaroscopic pattern could be easily followed up, and no targeted cohort study to achieve these ends has been developed. Such a cohort study is very much needed, but it would require documenting all appropriate clinical, genetic, and autoimmune measures, followed for at least 5 and perhaps more years, using a randomized menu of treatments.