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. 2022 Jul 6;61(7):2969-2977.
doi: 10.1093/rheumatology/keab846.

Association of anti-HSC70 autoantibodies with cutaneous ulceration and severe disease in juvenile dermatomyositis

Rie Karasawa  1 , Kazuo Yudoh  1 , Toshiko Sato  1 , Megumi Tanaka  1 , Mayumi Tamaki  1 , Sara E Sabbagh  2   3 , Terrance P O'Hanlon  4 , Payam Noroozi-Farhadi  4 , Ira N Targoff  5 , Willy A Flegel  6 , Andrew L Mammen  2 , Frederick W Miller  4 , Mark D Hicar  7 , Lisa G Rider  4 , James N Jarvis  7   8
Affiliations

Association of anti-HSC70 autoantibodies with cutaneous ulceration and severe disease in juvenile dermatomyositis

Rie Karasawa et al. Rheumatology (Oxford). .

Abstract

Objectives: JDM is an inflammatory myopathy characterized by prominent vasculopathy. AECAs are frequently detected in inflammatory and autoimmune diseases. We sought to determine whether AECAs correlate with clinical features of JDM, and thus serve as biomarkers to guide therapy or predict outcome.

Methods: Plasma samples from 63 patients with JDM, 49 patients with polyarticular JIA and 40 juvenile healthy controls were used to detect anti-heat shock cognate 71 kDa protein (HSC70) autoantibodies, a newly identified AECA, in ELISA assays. Clinical features were compared between JDM patients with and without anti-HSC70 autoantibodies.

Results: Anti-HSC70 autoantibodies were detected in 35% of patients with JDM, in 0% of patients with JIA (P < 0.0001) and in 0% of healthy donors (P < 0.0001). Both the presence of cutaneous ulcers (59% vs 17%, P < 0.002) and the use of wheelchairs and/or assistive devices (64% vs 27%, P < 0.007) were strongly associated with anti-HSC70 autoantibodies in JDM. High scores on the severity of myositis damage measures at the time of measurement of anti-HSC70 autoantibodies and an increased number of hospitalizations were also associated with anti-HSC70 autoantibodies. Intravenous immunoglobulin therapy was used more often in anti-HSC70 autoantibody-positive patients.

Conclusion: Anti-HCS70 autoantibodies are detected frequently in children with JDM and are novel myositis-associated autoantibodies correlating with disease severity.

Keywords: JDM; anti-endothelial cell antibodies; heat shock cognate 71 kDa protein; myositis-associated autoantibodies; vasculopathy.

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Figures

<sc>Fig.</sc> 1
Fig. 1
Results of ELISAs for anti-HSC70 autoantibodies The dashed line of 100 arbitrary units indicates the cut-off values for anti-HSC70 autoantibody positivity. The average plus 3 s.d. among the group of healthy donors defined as 100 arbitrary units was 0.603 (optical density). The continuous lines indicate median and interquartile range of anti-HSC70 autoantibody levels. Plasma samples from 63 patients with JDM, 49 patients with JIA, and 40 sex- and age-matched healthy children (HC) were used for ELISA assays. Anti-HSC70 autoantibodies were detected in 35% of patients with JDM, in 0% of patients with JIA, and in 0% of HC.
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