Clinical and Radiological Features of Korean Patients With Anti-HMGCR Myopathy
- Author:
Eun Kyoung OH
1
;
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
Author Information
- Publication Type:ORIGINAL ARTICLE
- From:Journal of Clinical Neurology 2023;19(5):460-468
- CountryRepublic of Korea
- Language:English
-
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.
