J Clin Neurol.
2023 Sep;19(5):460-468. 10.3988/jcn.2022.0374.
Clinical and Radiological Features of Korean Patients With Anti-HMGCR Myopathy
- Oh EK
1 - Lee SA2
- Lee HJ1
- Cha YJ3
- Kim S4
- Lee HS5
- Suh BC6
- Shin HY7
- Kim SW7
- Yoon BA8
- Oh Si9
- Kim YH10
- Cho JY11
- Cho JH12
- Kwon KH13
- Choi YC1,14
- Park HJ1,14
- Affiliations
-
- 1Departments of Neurology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- 2Department of Neurology, Mokdong Hospital, Ewha Womans University School of Medicine, Seoul, Korea
- 3Departments of Pathology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- 4Departments of Radiology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- 5Department of Neurology, Korea University Ansan Hospital, Ansan, Korea
- 6Department of Neurology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
- 7Department of Neurology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- 8Department of Neurology, Peripheral Neuropathy Research Center, College of Medicine, Dong-A University, Busan, Korea
- 9Department of Neurology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
- 10Department of Neurology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea
- 11Department of Neurology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea
- 12Department of Neurology, National Health Insurance Service Ilsan Hospital, Goyang, Korea
- 13Department of Neurology, Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea
- 14Rehabilitation Institute of Neuromuscular Disease, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- KMID: 2545499
- DOI: http://doi.org/10.3988/jcn.2022.0374
Abstract
- Background and Purpose
To understand the characteristics of Korean patients with anti-3-hydroxy-3-methylglutaryl-coenxyme A reductase (HMGCR) myopathy, we measured antiHMGCR 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 antiHMGCR 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.
