title: Anti-centromere antibody-positive Sjögren's syndrome: A distinct clinical subgroup?
abstract: OBJECTIVE
  To investigate whether patients with Sjögren's syndrome (SS) can be distinguished based on the positivity of anti-centromere antibody (ACA), and if so, whether the subgroups differ in their clinical and laboratory features.
  
  
  METHODS
  Eleven patients with ACA-positive and 71 patients ACA-negative SS were examined. All patients had minor salivary gland biopsy; sociodemographic data, glandular and extraglandular manifestations, and laboratory findings, including autoantibodies, complement and immunoglobulin levels, were analyzed. European League Against Rheumatism SS disease activity index (ESSDAI) and SS disease damage index (SSDDI) were also measured.
  
  
  RESULTS
  The prevalence of ACA among SS patients was 13.4%. ACA-positive SS patients had a higher prevalence of Raynaud's phenomenon, sclerodactyly and autoimmune thyroiditis and a lower prevalence of anti-SSA/Ro and anti-SSB/La antibodies compared to ACA-negative patients. Disease activity was higher in ACA-positive patients than in ACA-negative patients, but the damage index did not differ between the two groups. None of the patients who originally had ACA evolved to having full-blown systemic sclerosis.
  
  
  CONCLUSIONS
  Patients with SS who have ACA differ from classic SS patients in several clinical and laboratory parameters. ACA should be considered one of the pathogenically relevant autoantibodies for SS.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.
authors.forename: Kyung-Eun
authors.initials: KE
authors.lastname: Lee
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.
authors.forename: Ji-Hyoun
authors.initials: JH
authors.lastname: Kang
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.
authors.forename: Jeong-Won
authors.initials: JW
authors.lastname: Lee
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.
authors.forename: Lihui
authors.initials: L
authors.lastname: Wen
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.
authors.forename: Dong-Jin
authors.initials: DJ
authors.lastname: Park
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.
authors.forename: Tae-Jong
authors.initials: TJ
authors.lastname: Kim
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.
authors.forename: Yong-Wook
authors.initials: YW
authors.lastname: Park
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.
authors.forename: Shin-Seok
authors.initials: SS
authors.lastname: Lee
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D015995:Prevalence; D011379:Prognosis; D056910:Republic of Korea / Q000453:epidemiology; D012189:Retrospective Studies; D012469:Salivary Glands / Q000276:immunology* / Q000473:pathology; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000097:blood / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology*
chemical_list: D000974:Antibodies, Antinuclear; D015415:Biomarkers; C538950:anticentromere antibody
keywords: Sjögren's syndrome; anti-centromere antibody; autoantibodies