Pediatric rheumatology

Autoantibodies Recognizing Specificity Protein 4 Co‐occur With Anti–Transcription Intermediary Factor 1 and Are Associated With Distinct Clinical Features and Immunogenetic Risk Factors in Juvenile Myositis

Arthritis RheumatolPublished Cross-sectional study

Authors' conclusion

Anti-Sp4 autoantibodies were found in patients with juvenile-onset IIM, predominantly those with coexisting anti-TIF1 autoantibodies. Patients with anti-Sp4 autoantibodies represent a phenotypic subset of anti-TIF1 autoantibody-positive myositis characterized by frequent Raynaud's phenomenon and less pronounced muscle involvement, similar to adults with these autoantibodies.

Study context

Population
Patients with juvenile-onset idiopathic inflammatory myopathies (IIM) in a cross-sectional cohort and healthy controlsplus 91 healthy controls
Participants
336 patients
Exposure
Juvenile myositis with vs without anti-Sp4 autoantibodies
  • Anti-Sp4+n = 23
  • Healthy controlsn = 91
Outcome
Prevalence of anti-Sp4 autoantibodies and associated clinical features
Methodological detailsELISA screening
  • ELISA screening

Main finding

As reported in the abstract

Anti-Sp4 autoantibody prevalence (ELISA)

Juvenile myositis

7%

23 events

Observational data: associations, not effects.

Further findings

OutcomeAnti-Sp4+Anti-Sp4−P value
Anti-TIF1 autoantibody positivityAnti-Sp4+ vs Anti-Sp4−91%30%P < 0.001
RP (anti-TIF1+ subgroup)Anti-Sp4+ vs Anti-Sp4−38%2%P < 0.001
Wheelchair requirement0 events— not stated in the abstract

— not stated in the abstract

Peak aspartate aminotransferase (anti-TIF1+ subgroup)

Significantly lower with anti-Sp4 autoantibodies

HLA alleles (White patients)

DQA1*04 and DRB1*08 associated with anti-Sp4 autoantibodies

Original publication

Autoantibodies Recognizing Specificity Protein 4 Co‐occur With Anti–Transcription Intermediary Factor 1 and Are Associated With Distinct Clinical Features and Immunogenetic Risk Factors in Juvenile Myositis. Arthritis Rheumatol. Published 19 July 2023. doi:10.1002/art.42512

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