Autoantibodies to Dense Fine Speckles in Pediatric Diseases and Controls

J Rheumatol. 2015 Dec;42(12):2419-26. doi: 10.3899/jrheum.150567. Epub 2015 Oct 15.

Abstract

Objective: Autoantibodies to the dense fine speckled 70 kDa antigen (DFS70) are reported to be more common in individuals who do not have an antinuclear antibody (ANA)-associated rheumatic disease (AARD) than in patients with AARD. The frequency of anti-DFS70 antibodies has been thoroughly studied in adult but not in pediatric populations. The primary objective of this observational study was to determine the frequency of anti-DFS70 in pediatric AARD and reference cohorts.

Methods: Sera from 743 children with AARD and related conditions, and 345 samples from reference cohorts (healthy children and those being investigated for AARD) were studied for anti-DFS70 autoantibodies as measured by a chemiluminescence immunoassay. A de-identified administrative database was used to retrieve demographic, serologic, and clinical data.

Results: Anti-DFS70 antibodies were seen in 2.1% of healthy children and in 4.5% of sera from pediatric individuals referred for ANA testing. The frequency of anti-DFS70 was highest in juvenile localized scleroderma (LS; 4/29, 13.8%), juvenile dermatomyositis (JDM; 2/11, 18.2%), childhood systemic lupus erythematosus (cSLE; 19/331, 5.7%), diffuse cutaneous systemic sclerosis (1/22, 4.5%), celiac disease (2/49, 4.1%), and juvenile idiopathic arthritis (JIA; 5/202, 2.5%). Of note, anti-DFS70 antibodies were observed in 3/26 children (11.5%) with uveitis and JIA-associated uveitis.

Conclusion: The frequency of anti-DFS70 autoantibodies in healthy pediatric subjects is within the lower range of that reported in adults. Anti-DFS70 antibodies can be found in childhood SSc and cSLE, but has a remarkably high frequency in children with LS, JDM, and uveitis.

Keywords: ANTINUCLEAR ANTIBODIES; AUTOANTIBODIES; AUTOIMMUNE DISEASES; DENSE FINE SPECKLES; PEDIATRICS.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adaptor Proteins, Signal Transducing / immunology*
  • Adolescent
  • Age Factors
  • Antibodies, Antinuclear / blood
  • Antibodies, Antinuclear / immunology*
  • Autoantibodies / blood
  • Autoantibodies / immunology*
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / physiopathology
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Disease Progression
  • Female
  • Fluorescent Antibody Technique, Indirect / methods
  • Follow-Up Studies
  • Humans
  • Likelihood Functions
  • Male
  • Reference Values
  • Rheumatic Diseases / blood
  • Rheumatic Diseases / immunology*
  • Rheumatic Diseases / physiopathology
  • Risk Assessment
  • Sex Factors
  • Transcription Factors / immunology*

Substances

  • Adaptor Proteins, Signal Transducing
  • Antibodies, Antinuclear
  • Autoantibodies
  • PSIP1 protein, human
  • Transcription Factors