Anti-FHL1 autoantibodies in adult patients with myositis: a longitudinal follow-up analysis

Author:

Galindo-Feria Angeles S123ORCID,Lodin Karin123,Horuluoglu Begum12,Sarrafzadeh-Zargar Sepehr12,Wigren Edvard14,Gräslund Susanne14,Danielsson Olof5,Wahren-Herlenius Marie126ORCID,Dastmalchi Maryam123,Lundberg Ingrid E123ORCID, ,Mohammad Aladdin J,Leonard Dag,Sjöwall Christopher,Husmark Thomas,Ask Malin,Puksic Silva,Lappas Theodoros,Hanna Balsam

Affiliation:

1. Department of Medicine, Division of Rheumatology, Karolinska Institutet , Stockholm, Sweden

2. Center for Molecular Medicine, Division for Rheumatology, Department of Medicine, Solna, Karolinska Institutet , Stockholm, Sweden

3. Department of Gastro, Dermatology and Rheumatology, Theme Inflammation and Aging, Karolinska University Hospital , Stockholm, Sweden

4. Department of Medicine, Division of Rheumatology, Structural Genomics Consortium, Karolinska Institutet , Stockholm, Sweden

5. Department of Biomedical and Clinical Sciences, Division of Neurology, Faculty of Medicine and Health Sciences, Linkoping University , Linkoping, Sweden

6. Department of Clinical Science, Broegelmanns Research Laboratory, University of Bergen, Bergen , Norway

Abstract

Abstract Objectives To determine prevalence and clinical associations of anti-Four-and-a-half-LIM-domain 1 (FHL1) autoantibodies in patients with idiopathic inflammatory myopathies (IIM) and to evaluate autoantibody levels over time. Methods Sera at the time of diagnosis from patients with IIM (n = 449), autoimmune disease controls (DC, n = 130), neuromuscular diseases (NMDs, n = 16) and healthy controls (HC, n = 100) were analysed for anti-FHL1 autoantibodies by enzyme-linked immunosorbent assay (ELISA). Patients with IIM FHL1+ and FHL1− were included in a longitudinal analysis. Serum levels were correlated to disease activity. Results Autoantibodies to FHL1 were more frequent in patients with IIM (122/449, 27%) compared with DC (autoimmune DC and NMD, 13/146, 9%, P < 0.001) and HC (3/100.3%, P < 0.001). Anti-FHL1 levels were higher in IIM [median (IQR)=0.62 (0.15–1.04)] in comparison with DC [0.22 (0.08–0.58)], HC [0.35 (0.23–0.47)] and NMD [0.48 (0.36–0.80)] P < 0.001. Anti-FHL1+ patients with IIM were younger at the time of diagnosis compared with the anti-FHL1− group (P = 0.05) and were seronegative for other autoantibodies in 25%. In the first follow-up, anti-FHL1+ sample 20/33 (60%) positive at baseline had turned negative for anti-FHL1 autoantibodies. Anti-FHL1 autoantibodies rarely appeared after initiating treatment. Anti-FHL1 autoantibody levels correlated with CK (r = 0.62, P= 0.01), disease activity measured using the Myositis Disease Activity Assessment Tool (MYOACT) (n = 14, P = 0.004) and inversely with Manual Muscle Test-8 (r = −0.59, P = 0.02) at baseline. Conclusion Anti-FHL1 autoantibodies were present in 27% of patients with IIM; of these, 25% were negative for other autoantibodies. Other autoimmune diseases had lower frequencies and levels. Anti-FHL1 levels often decreased with immunosuppressive treatment, correlated with disease activity measures at diagnosis and rarely appeared after start of treatment.

Funder

Swedish Research Council

Swedish Rheumatism Association

King Gustaf V 80 Year Foundation

Konung Gustaf V: s och Drottning Victorias Frimurarestiftelse

Region Stockholm

Publisher

Oxford University Press (OUP)

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