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Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features.

Publication ,  Journal Article
Sherman, MA; Graf, R; Sabbagh, SE; Galindo-Feria, AS; Pinal-Fernandez, I; Pak, K; Kishi, T; Flegel, WA; Targoff, IN; Miller, FW; Lundberg, IE ...
Published in: Rheumatology (Oxford)
February 23, 2023

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.

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Published In

Rheumatology (Oxford)

DOI

EISSN

1462-0332

Publication Date

February 23, 2023

Volume

62

Issue

SI2

Start / End Page

SI226 / SI234

Location

England

Related Subject Headings

  • Myositis
  • Muscle Proteins
  • LIM Domain Proteins
  • Intracellular Signaling Peptides and Proteins
  • Humans
  • Exanthema
  • Dermatomyositis
  • Child
  • Autoantibodies
  • Arthritis & Rheumatology
 

Citation

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Sherman, M. A., Graf, R., Sabbagh, S. E., Galindo-Feria, A. S., Pinal-Fernandez, I., Pak, K., … Childhood Myositis Heterogeneity Collaborative Study Group, . (2023). Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features. Rheumatology (Oxford), 62(SI2), SI226–SI234. https://doi.org/10.1093/rheumatology/keac428
Sherman, Matthew A., Rose Graf, Sara E. Sabbagh, Angeles S. Galindo-Feria, Iago Pinal-Fernandez, Katherine Pak, Takayuki Kishi, et al. “Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features.Rheumatology (Oxford) 62, no. SI2 (February 23, 2023): SI226–34. https://doi.org/10.1093/rheumatology/keac428.
Sherman MA, Graf R, Sabbagh SE, Galindo-Feria AS, Pinal-Fernandez I, Pak K, et al. Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features. Rheumatology (Oxford). 2023 Feb 23;62(SI2):SI226–34.
Sherman, Matthew A., et al. “Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features.Rheumatology (Oxford), vol. 62, no. SI2, Feb. 2023, pp. SI226–34. Pubmed, doi:10.1093/rheumatology/keac428.
Sherman MA, Graf R, Sabbagh SE, Galindo-Feria AS, Pinal-Fernandez I, Pak K, Kishi T, Flegel WA, Targoff IN, Miller FW, Lundberg IE, Rider LG, Mammen AL, Childhood Myositis Heterogeneity Collaborative Study Group. Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features. Rheumatology (Oxford). 2023 Feb 23;62(SI2):SI226–SI234.
Journal cover image

Published In

Rheumatology (Oxford)

DOI

EISSN

1462-0332

Publication Date

February 23, 2023

Volume

62

Issue

SI2

Start / End Page

SI226 / SI234

Location

England

Related Subject Headings

  • Myositis
  • Muscle Proteins
  • LIM Domain Proteins
  • Intracellular Signaling Peptides and Proteins
  • Humans
  • Exanthema
  • Dermatomyositis
  • Child
  • Autoantibodies
  • Arthritis & Rheumatology