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

Abstract

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.

• Authors: 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: Journal of clinical neurology (Seoul, Korea) (J Clin Neurol) Vol. 19 Issue 5 Pg. 460-468 (Sep 2023) ISSN: 1738-6586 [Print] Korea (South)
• PMID: 36929062 (Publication Type: Journal Article)
• Copyright: Copyright © 2023 Korean Neurological Association.