Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Jun 1;64(6):3900-3905.
doi: 10.1093/rheumatology/keae574.

Anti-Sp4 and anti-CCAR1 autoantibodies in UK vs US patients with adult and juvenile-onset anti-TIF1γ-positive myositis

Fionnuala K McMorrow  1 , Lucy R Wedderburn  2   3   4 , Hector Chinoy  5   6 , Alexander Oldroyd  5   6 , Janine A Lamb  7 , Lisa G Rider  8 , Andrew L Mammen  9   10 , Livia Casciola-Rosen  10 , Neil J McHugh  1 , Sarah L Tansley  1   11 ; UK JDCBS and Childhood Myositis Heterogeneity Study Group
Collaborators, Affiliations

Anti-Sp4 and anti-CCAR1 autoantibodies in UK vs US patients with adult and juvenile-onset anti-TIF1γ-positive myositis

Fionnuala K McMorrow et al. Rheumatology (Oxford). .

Abstract

Objectives: Anti-transcriptional intermediary factor 1γ (TIF1γ) autoantibodies are associated with malignancy in adult-onset idiopathic inflammatory myopathy (IIM) and this risk is attenuated if patients are also positive for anti-specificity protein 4 (Sp4) or anti-cell division cycle apoptosis regulator protein 1 (CCAR1). In anti-TIF1γ positive dermatomyositis (DM) patients from the USA, anti-Sp4 and anti-CCAR1 autoantibody frequencies are reported as 32% and 43% in adults and 9% and 19% in juveniles, respectively. This study aims to identify the frequency of anti-Sp4 and anti-CCAR1 in adult and juvenile UK anti-TIF1γ-positive myositis populations and report clinical associations.

Methods: Serum samples from 51 UK participants with adult-onset IIM and 55 UK participants with JDM, all anti-TIF1γ autoantibody positive, and 24 healthy control samples were screened for anti-Sp4 and anti-CCAR1 autoantibodies by ELISA.

Results: In UK adult anti-TIF1γ positive IIM patients, anti-Sp4 and anti-CCAR1 frequencies were 4% (2/51) and 16% (8/51). Both adult patients with anti-Sp4 were also positive for anti-CCAR1. In UK juveniles, anti-Sp4 was not detected and 13% (7/55) had anti-CCAR1 autoantibodies. Nineteen (37%) anti-TIF1γ positive UK adult myositis patients had cancer; neither of the two patients with anti-Sp4 autoantibodies and 25% (2/8) of anti-CCAR1 autoantibody-positive patients had cancer. No anti-Sp4 or anti-CCAR1 clinical associations were identified.

Conclusion: Anti-Sp4 and anti-CCAR1 autoantibodies are less common in the adult UK anti-TIF1γ-positive myositis population compared with published data from the USA, limiting their use as biomarkers for cancer risk. In patients with juvenile onset disease, anti-Sp4 is less frequent in UK patients compared with the USA, but the prevalence of anti-CCAR1 autoantibodies is similar.

Keywords: Juvenile dermatomyositis; autoantibodies; cancer; dermatomyositis; myositis.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Anti-Sp4 and anti-CCAR1 frequency in anti-TIF1γ positive UK adult and juvenile myositis cohorts compared with previously published data on cohorts from the USA. (A) Percentage of anti-Sp4. (B) Percentage of anti-CCAR1. US results previously published. References for (A): adult myositis [8], juvenile DM [11]. References for (B): adult myositis [10], juvenile DM [12]. Antibody frequency compared using Fisher’s exact test, *P ≤ 0.05, ****P ≤0.0001. CCAR1: cell division cycle apoptosis regulator protein 1; DM: dermatomyositis; Sp4: specificity protein 4; TIF1γ: transcriptional intermediary factor 1γ
See this image and copyright information in PMC

References

    1. Trallero-Araguás E, Rodrigo-Pendás JÁ, Selva-O'Callaghan A et al. Usefulness of anti-p155 autoantibody for diagnosing cancer-associated dermatomyositis: a systematic review and meta-analysis. Arthritis Rheum 2012;64:523–32. - PubMed
    1. Tiniakou E, Mammen AL. Idiopathic inflammatory myopathies and malignancy: a comprehensive review. Clin Rev Allergy Immunol 2017;52:20–33. - PubMed
    1. Oldroyd AGS, Callen JP, Chinoy H et al.; International Myositis Assessment and Clinical Studies Group Cancer Screening Expert Group. International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening: an International Myositis Assessment and Clinical Studies Group (IMACS) initiative. Nat Rev Rheumatol 2023;19:805–17. - PMC - PubMed
    1. Tansley SL, Simou S, Shaddick G et al. Autoantibodies in juvenile-onset myositis: their diagnostic value and associated clinical phenotype in a large UK cohort. J Autoimmun 2017;84:55–64. - PMC - PubMed
    1. Rider LG, Shah M, Mamyrova G et al.; Childhood Myositis Heterogeneity Collaborative Study Group. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore) 2013;92:223–43. - PMC - PubMed

MeSH terms