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Clinical features of Japanese systemic sclerosis (SSc) patients negative for SSc‐related autoantibodies: A single‐center retrospective study
Author(s) -
Miyake Miho,
Matsushita Takashi,
Takehara Kazuhiko,
Hamaguchi Yasuhito
Publication year - 2020
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.13908
Subject(s) - iif , medicine , autoantibody , anti nuclear antibody , scleroderma (fungus) , interstitial lung disease , incidence (geometry) , pathology , gastroenterology , antibody , immunology , lung , physics , inoculation , optics
Objective To examine the clinical features of systemic sclerosis (SSc) patients negative for SSc‐related autoantibodies (autoAbs). Methods Serum samples were collected from 546 SSc patients. The presence of antinuclear antibody (ANA) was screened by indirect immunofluorescence (IIF) staining using HEp‐2 cells. SSc‐related autoantibodies were identified by specific IIF staining, enzyme‐linked immunosorbent assay, or immunoprecipitation assay. Clinical features were analyzed 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. Results 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 features, patients negative for ANA/SSc‐related autoAbs (n = 55) had a significantly shorter disease duration, higher proportion 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). 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 features were similar. Conclusion Patients negative for ANA/SSc‐related autoAbs form a clinically distinct subset among SSc patients.