Abstract

BackgroundTo investigate the association between myositis autoantibodies and clinical subsets of inflammatory myositis in Korean patients.MethodsImmunoprecipitation was performed using the sera of classic polymyositis (PM) (n = 11) and dermatomyositis (DM) (n = 38) patients who met the Bohan and Peter criteria for definite inflammatory myositis. A panel of defined myositis autoantibodies was surveyed to investigate the association between each autoantibody and clinical subsets of inflammatory myositis.ResultsEither MSAs, anti-p140, or anti-p155/140 antibodies were found in 63.3% (31/49) of the study subjects. Anti-140-kDa-polypeptide (anti-p140) (18.4%, 9/49) and anti-155/140-kDa polypeptide (anti-p155/140) (16.3%, 8/49) antibodies were the most common, followed by anti-Mi2 (14.3%, 7/49), anti-ARS (12.2%, 6/49) and anti-SRP (2.0%, 1/49) antibodies. All MSAs and anti-p140 and anti-p155/140 antibodies were mutually exclusive. Anti-p140 (23.7%, 9/38), anti-p155/140 (21.1%, 8/38), and anti-Mi2 (18.4%, 3/38) antibodies were found exclusively in DM patients. Anti-p140 antibody was associated with rapidly progressive interstitial lung disease (ILD) (p = 0.001), with a sensitivity of 100.0% (4/4) and a specificity of 85.3% (29/34) in DM patients. Anti-p155/140 antibody was associated with cancer-associated DM (p = 0.009), with a sensitivity of 55.6% (5/9) and a specificity of 89.7% (26/29). Cancer-associated survival was significantly worse when anti-p155/140 antibody was present (19.2 ± 7.6 vs. 65.0 ± 3.5 months, p = 0.032). Finally, anti-ARS antibodies were associated with stable or slowly progressive ILD in PM and DM patients (p = 0.005).ConclusionsAnti-p140 and anti-p155/140 antibodies were commonly found autoantibodies in Korean patients with inflammatory myositis. Despite the lack of clinically amyopathic DM patients in the study subjects, a strong association was observed between anti-p140 antibody and rapidly progressive ILD. Anti-p155/140 antibody was associated with cancer-associated myositis and poor survival.

Highlights

  • To investigate the association between myositis autoantibodies and clinical subsets of inflammatory myositis in Korean patients

  • We investigated the panel of defined autoantibodies including MSAs, MAAs, antip140, and anti-p155/140 antibodies in the sera of Korean inflammatory myositis patients with the intention to classify clinical subsets of these patients based on the presence of myositis autoantibodies and to refine the relationships between these antibodies and disease manifestations

  • All rapidly progressive interstitial lung disease (ILD) developed in DM patients (n = 4)

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Summary

Introduction

To investigate the association between myositis autoantibodies and clinical subsets of inflammatory myositis in Korean patients. Polymyositis (PM) and dermatomyositis (DM) are systemic autoimmune diseases in which muscles are the primary target of immune-mediated inflammation. Amyopathic dermatomyositis (ADM) is a condition in which the typical skin manifestations of DM develop without muscle involvement, and it constitutes the clinical spectrum of inflammatory myositis together with PM and DM [4]. Amyopathic dermatomyositis (CADM) is an extended concept of ADM in which no muscle weakness is observed with or without subclinical evidence of muscle inflammation on laboratory, electrophysiological, and/or radiographic evaluations [5]. Treatment-resistant rapidly progressive interstitial lung disease (ILD) has been reported to cluster in ADM/CADM patients [5,6,7], and appreciable clinical significance has been conferred upon ADM and/or CADM (ADM/CADM)

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