Putative functional pathogenic autoantibodies in systemic sclerosis

Abstract

Systemic sclerosis (scleroderma, SSc) is a systemic disease characterized by vascular lesions, fibrosis,and circulating autoantibodies. A complex interplay between innate and adaptive immunity, andwith regard to the latter, between humoral and cellular immunity, is believed to be involved in SScpathogenesis. Lately, close attention has been paid to the role of B cells which, once activated, releaseprofibrotic cytokines, promote profibrotic Th2 differentiation, and produce autoantibodies. Severalnovel interesting autoantibodies, targeting antigens within the extracellular matrix or on the cell surface,rather than the nuclear antigens of canonical SSc-autoantibodies, have been recently describedin patients with SSc. As they show stimulatory or inhibitory activity or react with structures involvedin the pathogenesis of SSc lesions, they can be considered as potentially pathogenic. In this paper, wewill review those which have been better characterized.