{"doi":"10.1002/art.42768","title":"Clinical Features and Immunogenetic Risk Factors Associated With Additional Autoantibodies in Anti‐Transcriptional Intermediary Factor 1γ <scp>Juvenile‐Onset</scp> Dermatomyositis","abstract":"OBJECTIVE: Novel autoantibody specificities including anti-CCAR1 were recently discovered in adult patients with anti-transcriptional intermediary factor (TIF1)-positive dermatomyositis (DM) and were associated with attenuated cancer emergence. The aims of the present study were to examine whether these autoantibodies occur in patients with juvenile-onset DM (JDM) and to determine their associated features. METHODS: Sera from 150 patients with anti-TIF1γ autoantibody-positive JDM in a cross-sectional cohort and 90 juvenile healthy controls were assayed for anti-CCAR1, anti-C1Z1, anti-IMMT, anti-TBL1XR1, and anti-Sp4 autoantibodies. Demographics, myositis autoantibodies, clinical features, medications, outcomes, and HLA-DRB1 and HLA-DQA1 alleles were compared between those with and without these autoantibodies. RESULTS: Any one of the anti-TIF1γ-associated autoantibodies was present in 44 patients (29%) overall, including 25 (17%) with anti-Sp4, 22 (15%) with anti-TBL1XR1, 14 (9%) with anti-CCAR1, 2 (1%) with anti-C1Z1, and 2 (1%) with anti-IMMT autoantibodies. These anti-TIF1γ-associated autoantibodies frequently co-occurred. Patients with any of the anti-TIF1γ-associated autoantibodies had less frequent falling (34% [15] vs. 53% [56], P = 0.032) and lower peak muscle enzymes. None of the patients had cancer. Among White patients, HLA-DRB1*03 was protective against an anti-TIF1γ-associated autoantibody (odds ratio 0.20, 95% confidence interval 0.07-0.52). CONCLUSION: Autoantibodies associated with anti-TIF1γ were found in isolation and in combination among a subset of patients with JDM. Patients with these autoantibodies had less severe muscle disease and were not enriched for HLA-DRB1*03. Additional autoantibodies among patients with positive anti-TIF1γ with JDM likely contribute to the heterogeneity of the anti-TIF1γ serologic subgroup.","journal":"Arthritis & Rheumatology","year":2023,"id":366509,"datarank":0.29188652235829704,"base_score":1.9459101490553132,"endowment":1.9459101490553132,"self_citation_contribution":0.29188652235829704,"citation_network_contribution":0.0,"self_endowment_contribution":0.29188652235829704,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":6,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9564,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2023-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1030979,"name":"Qingyuan Yang","orcid":"0000-0001-8808-1113","position":1,"is_corresponding":false},{"id":260832,"name":"Laura Gutierrez‐Alamillo","orcid":null,"position":2,"is_corresponding":false},{"id":260868,"name":"Katherine Pak","orcid":null,"position":3,"is_corresponding":false},{"id":347479,"name":"Willy A. Flegel","orcid":"0000-0002-1631-7198","position":4,"is_corresponding":false},{"id":257669,"name":"Andrew L. Mammen","orcid":"0000-0003-3732-3252","position":5,"is_corresponding":false},{"id":264511,"name":"Lisa G. Rider","orcid":"0000-0002-6912-2458","position":6,"is_corresponding":false},{"id":257451,"name":"Livia Casciola‐Rosen","orcid":"0000-0002-4172-1539","position":7,"is_corresponding":false},{"id":327466,"name":"for the Childhood Myositis Heterogeneity Collaborative Study Group","orcid":null,"position":8,"is_corresponding":false},{"id":972343,"name":"Matthew A. Sherman","orcid":"0000-0002-5448-1538","position":0,"is_corresponding":true}],"reference_count":24,"raw_metadata":{"citation_network_status":"fetched"},"created_at":"2026-07-19T01:14:55.198227Z","pmid":"38059274","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}