Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features
Author:
Sherman Matthew A1ORCID, Graf Rose1, Sabbagh Sara E2, Galindo-Feria Angeles S34ORCID, Pinal-Fernandez Iago15, Pak Katherine1, Kishi Takayuki6ORCID, Flegel Willy A7, Targoff Ira N8, Miller Frederick W6, Lundberg Ingrid E34ORCID, Rider Lisa G6ORCID, Mammen Andrew L159, Albert Daniel A, Arabshahi Bita, Balboni Imelda M, Ballinger Susan, Barillas-Arias Lilliana, Becker Mara L, Bingham C April, Bohnsack John F, Carrasco Ruy, Cartwright Victoria W, Cron Randy Q, Curiel Rodolfo, Dare Jason A, de la Pena Wendy, DeGuzman Marietta M, Eberhard B Anne, Edelheit Barbara S, Finkel Terri H, George Stephen W, Gewanter Harry L, Goldmuntz Ellen A, Groh Brandt P, Haftel Hillary H, Hannan William P, Henrickson Michael, Higgins Gloria C, Hobday Patricia M, Hopp Russell J, Huber Adam M, Imundo Lisa, Inman C J, Jansen Anna, Jarvis James, Jones Olcay Y, Kamdar Ankur, Kim Hanna, Kingsbury Daniel J, Lindsley Carol B, Mamyrova Gulnara, McCarthy Paul L, Mitchell Stephen R, Murphy Frederick T, Nanda Kabita, O’Hanlon Terrance, Oral Elif A, Pachman Lauren M, Perez Maria D, Person Donald A, Rabinovich C Egla, Ronis Tova, Schiffenbauer Adam, Shaham Bracha, Sinal Sara H, Soep Jennifer, Stoll Matthew L, Sule Sangeeta, Tarvin Stacey, Vogelgesang Scott A, Volochayev Rita, Wargula Jennifer C, White Patience H,
Affiliation:
1. Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health , Bethesda, MD 2. Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin , Milwaukee, Wisconsin, USA 3. Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet 4. Center for Molecular Medicine, Karolinska Institutet, Karolinska University Hospital Solna , Stockholm, Sweden 5. Department of Neurology, Johns Hopkins University School of Medicine , Baltimore 6. Environmental Autoimmunity Group, National Institute of Environmental Health Sciences 7. Department of Transfusion Medicine, NIH Clinical Center, National Institutes of Health , Bethesda, MD 8. Veteran's Affairs Medical Center, University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation , Oklahoma City, OK 9. Department of Medicine, Johns Hopkins University School of Medicine , Baltimore, MD, USA
Abstract
Abstract
Objectives
Four-and-a-half LIM domains 1 (FHL1) is a muscle-specific protein. Autoantibodies against FHL1 were recently discovered in adults with idiopathic inflammatory myopathies (IIMs) and were found to be associated with clinical features and outcomes indicative of increased disease severity. Anti-FHL1 autoantibodies have not been described in children. Here, the prevalence and clinical features associated with anti-FHL1 autoantibodies were examined in a large North American cohort of juvenile patients with IIM.
Methods
Sera from 338 juvenile IIM patients and 91 juvenile healthy controls were screened for anti-FHL1 autoantibodies by ELISA. Clinical characteristics and HLA alleles of those with and without anti-FHL1 autoantibodies were compared among those with juvenile IIM.
Results
Anti-FHL1 autoantibodies were present in 10.9% of juvenile IIM patients and 1.1% of controls. The frequency of anti-FHL1 autoantibodies among clinical and serologic subgroups did not differ. A higher percentage of Asian patients had anti-FHL1 autoantibodies (11% vs 0.7%; P = 0.002). Myositis-associated autoantibodies (MAAs) [odds ratio (OR) 2.09 (CI 1.03, 4.32)], anti-Ro52 autoantibodies specifically [OR 4.17 (CI 1.83, 9.37)] and V-sign rash [OR 2.59 (CI 1.22, 5.40)] were associated with anti-FHL1 autoantibodies. There were no differences in other features or markers of disease severity. No HLA associations with anti-FHL1 autoantibodies in Caucasian myositis patients were identified.
Conclusion
Anti-FHL1 autoantibodies are present in ∼11% of juvenile IIM patients and commonly co-occur with MAAs, including anti-Ro52 autoantibodies. In contrast to adult IIM, anti-FHL1 autoantibodies in juvenile myositis are associated with V-sign rash but not with other distinctive clinical features or worse outcomes.
Funder
National Institute of Arthritis and Musculoskeletal and Skin Diseases National Institute of Environmental Health Sciences Clinical Center National Institutes of Health Cure JM Foundation Swedish Research Council King Gustaf V 80-year Foundation Swedish Rheumatism Association Stockholm Region Council
Publisher
Oxford University Press (OUP)
Subject
Pharmacology (medical),Rheumatology
Reference22 articles.
1. Idiopathic inflammatory myopathies;Lundberg;Nat Rev Dis Primers,2021 2. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies;Rider;Medicine (Baltimore),2013 3. Anti-Ro52 autoantibodies are associated with interstitial lung disease and more severe disease in patients with juvenile myositis;Sabbagh;Ann Rheum Dis,2019 4. Development of autoantibodies against muscle-specific FHL1 in severe inflammatory myopathies;Albrecht;J Clin Invest,2015 5. Autoantibodies against four-and-a-half-LIM domain 1 (FHL1) in inflammatory myopathies: results from an Australian single-center cohort;Galindo-Feria;Rheumatology (Oxford),2022
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