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Clinical and Radiological Features of Korean Patients With Anti-HMGCR Myopathy

 Eun Kyoung Oh  ;  Seung-Ah Lee  ;  Hyun Joon Lee  ;  Yoon Jin Cha  ;  Sungjun Kim  ;  Hyung-Soo Lee  ;  Bum Chun Suh  ;  Ha Young Shin  ;  Seung Woo Kim  ;  Byeol-A Yoon  ;  Seong-Il Oh  ;  Yoo Hwan Kim  ;  Joong-Yang Cho  ;  Jeong Hee Cho  ;  Ki-Han Kwon  ;  Young-Chul Choi  ;  Hyung Jun Park 
 JOURNAL OF CLINICAL NEUROLOGY, Vol.19(5) : 460-468, 2023-09 
Journal Title
Issue Date
HMGCR ; autoantibodies ; enzyme-linked immunosorbent assay ; myositis ; necrotizing myopathy
Background and Purpose To understand the characteristics of Korean patients with anti-3-hydroxy-3-methylglutaryl-coenxyme A reductase (HMGCR) myopathy, we measured anti-HMGCR antibodies and analyzed the clinical, radiological, and pathological features of patients with anti-HMGCR myopathy. Methods We measured titers of anti-HMGCR antibodies in the sera of 99 patients with inflammatory myopathy, 36 patients with genetic myopathy, and 63 healthy subjects using an enzyme-linked immunosorbent assay. We tested 16 myositis-specific autoantibodies (MSAs) in all patients with anti-HMGCR myopathy. Results Positivity for the anti-HMGCR antibody was observed in 17 (4 males and 13 females) of 99 patients with inflammatory myopathy. The median age at symptom onset was 60 years. Ten (59%) of the patients with anti-HMGCR positivity had taken statins. The titer of anti-HMGCR antibodies was significantly higher in the statin-naïve group (median=230 U/mL, interquartile range=170–443 U/mL) than in the statin-exposed group (median=178 U/mL, interquartile range=105–210 U/mL, p=0.045). The most common symptom was proximal muscle weakness in 15 patients (88%), followed by myalgia in 9 (53%), neck weakness in 4 (24%), dysphagia in 3 (18%), and skin lesions in 2 (12%). The median titer of anti-HMGCR antibody was 202 U/mL. We found eight different MSAs in nine (53%) patients. The median disease duration from symptom onset to diagnosis was significantly shorter in the MSA-positive group than in the MSA-negative group (p=0.027). Conclusions Our study was the first to measure anti-HMGCR antibodies in inflammatory myopathy. It has provided new findings, including the suggestion of the coexistence of other MSAs in Korean patients. © 2023 Korean Neurological Association.
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1. College of Medicine (의과대학) > Dept. of Neurology (신경과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Sungjun(김성준) ORCID logo https://orcid.org/0000-0002-7876-7901
Kim, Seung Woo(김승우) ORCID logo https://orcid.org/0000-0002-5621-0811
Park, Hyung Jun(박형준)
Shin, Ha Young(신하영) ORCID logo https://orcid.org/0000-0002-4408-8265
Cha, Yoon Jin(차윤진) ORCID logo https://orcid.org/0000-0002-5967-4064
Choi, Young Chul(최영철) ORCID logo https://orcid.org/0000-0001-5525-6861
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