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Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-127-32. Epub 2013 Oct 21.

Prevalence and clinical profiles of 'autoantibody-negative' systemic sclerosis subjects.

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  • 1Division of Rheumatology, Lady Davis Institute, Jewish General Hospital, Montreal, Canada.



To determine the prevalence of autoantibody negative systemic sclerosis (SSc) and to identify the clinical correlates thereof.


Clinical data and sera from 874 SSc subjects were collected and autoantibodies were tested in a central laboratory using 1) indirect immunofluorescence (IIF), 2) commercially available ELISA, addressable laser bead immunoassay (ALBIA), and line immunoassay (LIA), and 3) a sensitive immunoprecipitation (IP) assay.


Fifteen (15; 1.7%) subjects were autoantibody negative by IIF, ELISA, ALBIA, LIA and IP, and 16 (1.8%) were antinuclear antibody (ANA) positive by IIF but otherwise negative by ELISA, ALBIA, LIA and IP. Thirty-seven (37; 4.2%) were ANA positive by IIF, autoantibody negative by commercially available immunoassays, but had autoantibodies identified by IP (including Th/To in 20). Autoantibody-negative subjects had generally less severe disease than positive subjects.


Autoantibody-negative SSc is rare (<2%) and appears to be associated with a favourable prognosis.

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