Abstract

Objective This study aimed to clarify the clinical features, the serum level of autoantibodies, and cytokine of myositis patients with anti-EJ antibody, which targets glycyl tRNA-synthetase (GlyRS). Methods Sera of 236 Chinese patients with myositis were screened for anti-EJ by a novel immunoprecipitation assay of flag-tagged GlyRS. Anti-EJ positive patients are evaluated for the clinical features and cytokine profile. Results The sera from 4 of 236 adult myositis patients were found to carry the anti-EJ using established novel immunoprecipitation assay and immunoblotting. The prevalence of anti-EJ in our cohorts is about 1.7%. The decline of anti-EJ level was detected in two patients during disease remission. Interstitial lung disease and muscle weakness, but not skin involvement, are common clinical features of anti-EJ positive patients. Moreover, using a cytokine profile analyses, we found that the serum levels of IP-10, IL-6, MCP-1, and VEGF were significantly elevated in patients with anti-EJ and gradually decreased during disease remission of two patients, whereas IL-8 level was obviously reduced in these patients. Conclusion The novel immunoprecipitation assay is suitable to detect and monitor the levels of anti-EJ autoantibody. The serum levels of anti-EJ, IP-10, IL-6, MCP-1, and VEGF may be related to disease activity in myositis patients with anti-EJ antibodies.

Highlights

  • Antisynthetase syndrome (ASS) has been characterized as the clinical combination of myositis, interstitial lung disease (ILD), fever, arthritis, Raynaud’s phenomenon (RP), and Mechanic’s hands with the presence of anti-aminoacyl-tRNAsynthetase antibodies [1]

  • Our report retrospectively screened anti-EJ antibody in sera from 236 Chinese patients with idiopathic inflammatory myopathies (IIM) by established immunoprecipitation assay and four patients were positive for anti-EJ, A

  • The immunoprecipitation assay of flag-tagged glycyl tRNA-synthetase (GlyRS) overexpressed in HEK293 cells was suitable to detect the decline of anti-EJ autoantibody level in longitudinal sera from two patients during disease remission, which is consistent with the improved muscle weakness and normal muscle enzymes level

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Summary

Objective

This study aimed to clarify the clinical features, the serum level of autoantibodies, and cytokine of myositis patients with anti-EJ antibody, which targets glycyl tRNA-synthetase (GlyRS). Sera of 236 Chinese patients with myositis were screened for anti-EJ by a novel immunoprecipitation assay of flag-tagged GlyRS. Anti-EJ positive patients are evaluated for the clinical features and cytokine profile. The sera from 4 of 236 adult myositis patients were found to carry the anti-EJ using established novel immunoprecipitation assay and immunoblotting. The decline of anti-EJ level was detected in two patients during disease remission. Using a cytokine profile analyses, we found that the serum levels of IP-10, IL-6, MCP-1, and VEGF were significantly elevated in patients with anti-EJ and gradually decreased during disease remission of two patients, whereas IL-8 level was obviously reduced in these patients. The serum levels of anti-EJ, IP-10, IL-6, MCP-1, and VEGF may be related to disease activity in myositis patients with anti-EJ antibodies

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Materials and Methods
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