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- W4366178036 abstract "Although anti-nuclear antibody (ANA) is positive in more than 90% of systemic sclerosis (SSc) patients, approximately 5-10 % of SSc patients are negative for ANA or SSc-related autoAbs. This study aims to examine the clinical profiles of Japanese SSc patients negative for ANA or SSc-related autoAbs. The presence of ANA was screened by indirect immunofluorescence (IIF) staining using HEp-2 cells. SSc-related autoAbs were identified by specific IIF staining, enzyme-linked immunosorbent assay, and/or immunoprecipitation assay. We analyzed clinical profiles among patients negative for ANA/SSc-related autoAbs, anticentromere Abs (ACA), anti-topoisomerase I (anti-topo I) Abs, and anti-RNA polymerase (anti-RNAP) Abs. Of the 546 SSc patients, 26 (4.8%) were negative for ANA and 29 (5.3%) were ANA-positive but negative for SSc-related autoAbs. Regarding clinical profiles, patients negative for ANA/SSc-related autoAbs (n = 55) had a significantly shorter disease duration, higher frequency of the diffuse type, contracture of phalanges, diffuse pigmentation, higher modified Rodnan total skin thickness score (mRSS), and lower incidence of telangiectasia than those with ACA (n = 224). In addition, the percentage of patients receiving oral corticosteroid was significantly higher among those negative for ANA/SSc-related autoAbs than those with ACA. On the other hand, younger disease onset, lower mRSS, and lower incidence of scleroderma renal crisis were observed in patients negative for ANA/SSc-related autoAbs than in those with anti-RNAP Abs (n = 52). Although pitting scars were less common in patients negative for ANA/SSc-related autoAbs than in those with anti-topo I Abs (n = 144), their clinical profiles were similar. Thus, patients negative for ANA/SSc-related autoAbs form a clinically distinct subset among SSc patients." @default.
- W4366178036 created "2023-04-19" @default.
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- W4366178036 date "2023-05-01" @default.
- W4366178036 modified "2023-09-28" @default.
- W4366178036 title "422 Clinical profiles of systemic sclerosis (SSc)-related autoantibody-negative Japanese SSc patients" @default.
- W4366178036 doi "https://doi.org/10.1016/j.jid.2023.03.428" @default.
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