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

McMorrow, Fionnuala K; Wedderburn, Lucy R; Chinoy, Hector; Oldroyd, Alexander; Lamb, Janine A; Rider, Lisa G; Mammen, Andrew L; ... Tansley, Sarah L; + view all (2025) Anti-Sp4 and anti-CCAR1 autoantibodies in UK vs US patients with adult and juvenile-onset anti-TIF1γ-positive myositis. Rheumatology , 64 (6) pp. 3900-3905. 10.1093/rheumatology/keae574. Green open access

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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.

Type: Article
Title: Anti-Sp4 and anti-CCAR1 autoantibodies in UK vs US patients with adult and juvenile-onset anti-TIF1γ-positive myositis
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/rheumatology/keae574
Publisher version: https://doi.org/10.1093/rheumatology/keae574
Language: English
Additional information: © The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
Keywords: Science & Technology, Life Sciences & Biomedicine, Rheumatology, myositis, cancer, autoantibodies, dermatomyositis, Juvenile dermatomyositis, DERMATOMYOSITIS, PROTEIN
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10209636
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