Autoantibodies: A Marker for Scleroderma Progression?

Giovannetti A, Maselli A, Colasanti T, et al. 2013 Autoantibodies to Estrogen Receptor α in Systemic Sclerosis (SSc) as Pathogenetic Determinants and Markers of Progression, PLoS One; (2013) doi: 10.1371/journal.pone.0074332. First published online: September 18, 2013 (open access).

Autoantibodies to estrogen receptor α (anti-ERα) may trigger immune responses in systemic sclerosis (SSc), serving as a potential marker of scleroderma progression, Italian researchers say.

The study of anti-ERα antibodies in 71 consecutive SSc patients reveals serum immunoreactivity in 42% of the group (n=30) - but none among 90 matched healthy serum donor controls - plus a significant association between anti-ERα antibody values and key clinical parameters of disease activity and severity.

Among the SSc patients - predominantly women in their mid-50s with a median disease duration of 8 years - anti-ERα antibodies are twice as prevalent in those with a higher European Scleroderma Study Group (EScSG) activity index, compared with anti-ERα- negative patients. 

The study also finds anti-ERα serum antibodies are associated with diffuse SSc, anti-topoisomerase I antibodies (anti-Scl70 antibodies), and late capillaroscopic pattern in the disease.

Additionally, anti-ERα antibody positivity may be linked to greater susceptibility to apoptosis among T cells, leading to an autoantigen overload that initiates or perpetuates an autoimmune response and autoantibody-mediated tissue damage in diseases such as SSc, the researchers write.

Finally, the authors note that apoptotic T cells can trigger release of certain cytokines that play a key role in the pathogenesis of SSc.