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KMID : 1130620230190050460
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Journal of Clinical Neurology
2023 Volume.19 No. 5 p.460 ~ p.468
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Clinical and Radiological Features of Korean Patients With Anti-HMGCR Myopathy
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Oh Eun-Kyoung
Lee Seung-Ah
Lee Hyun-Joon
Cha Yoon-Jin
Kim Sung-Jun
Lee Hyung-Soo
Suh Bum-Chun
Shin Ha-Young
Kim Seung-Woo
Yoon Byeol-A
Oh Seong-Il
Kim Yoo-Hwan
Cho Joong-Yang
Cho Jeong-Hee
Kwon Ki-Han
Choi Young-Chul
Park Hyung-Jun
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Abstract
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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-naive 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.
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KEYWORD
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myositis, necrotizing myopathy, autoantibodies, HMGCR, enzyme-linked immunosorbent assay
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