Scleroderma: the role of serum autoantibodies in defining specific clinical phenotypes and organ system involvement

To discuss recent advances in serologic testing for systemic sclerosis (SSc)-associated antibodies with respect to the diagnosis and prognosis of the disease.

The importance of SSc antibodies for diagnosis has become increasingly recognized, as evidenced by incorporation into the 2013 American College of Rheumatology/the European League Against Rheumatism clinical classification criteria for SSc. Two new SSc-associated antibodies and their clinical associations have been described. Multiple cohort studies have reported variable antibody frequency distribution based on geography, but the clinical associations remain much the same. New associations include anti-RNA polymerase III antibodies with gastric antral vascular ectasia, and a temporal association between SSc onset and RNA polymerase III antibodies.

The role and associations of SSc-associated antibodies for diagnosis and internal organ involvement are becoming increasingly accepted.