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Statin-naïve anti-HMGCR antibody-mediated necrotizing myopathy in China.
J Clin Neurosci. 2018 Nov; 57:13-19.JC

Abstract

This study aimed to clarify the phenotypes and therapeutic responses of statin-naïve anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibody-mediated necrotizing myopathy. Anti-HMGCR antibodies were tested with ELISA methodology in the sera sample of 98 patients meeting the idiopathic inflammatory myopathy criteria and with negative anti-signal recognition particle (SRP) antibody. Twenty-one statin-naïve patients with anti-HMGCR antibody were detected (21.4%), with onset age from 6 to 67 years old. Proximal weakness and neck flexion weakness was the core neurological feature. The average maximal creatine kinase (CK) level was 7968.6 ± 4408.7U/L. Muscle MR imaging showed edema (88.2%), moderate or severe fatty replacement (70.6%) and muscle atrophy (88.2%) in lower limbs. Fatty replacement was significantly more prominent in the medial and posterior musculature than the anterior musculature (p = 0.0013). Seven (33.3%) patients were treated with mono-glucocorticoid, and thirteen (61.9%) patients needed adjuvant immunosuppressant. Eight (38.1%) patients experienced symptom relapse. The early-onset patients (<50 years old) were found with higher CK levels, shorter duration course, poorer response to adjuvant immunosuppressant and more recurrent weakness than the late-onset patients (≥50 years old). As a conclusion, Statin-naïve anti-HMGCR antibody-mediated necrotizing myopathy may not be rare. Compared with late-onset statin-naïve patients with anti-HMGCR antibody-mediated necrotizing myopathy, early-onset patients presented severer clinical features and worse therapeutic responses.

Authors+Show Affiliations

Department of Neurology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China.Department of Neurology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China.Department of Neurology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China.Department of Neurology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China.Department of Neurology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China.Department of Radiology, Jing'an District Center Hospital of Shanghai, No. 259 Xikang Road, Shanghai, China.Department of Neurology, Jing'an District Center Hospital of Shanghai, No. 259 Xikang Road, Shanghai, China.Department of Traditional Chinese Medicine, Jing'an District Center Hospital of Shanghai, No. 259 Xikang Road, Shanghai, China.Department of Clinical Electrophysiology, Institute of Neurology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China.Department of Pathology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China.Department of Neurology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China.Department of Neurology, Huashan Hospital, Fudan University, No. 12 Wulumuqi Road, Shanghai, China. Electronic address: lujiahong@fudan.edu.cn.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

30205933

Citation

Jiao, Yuqiong, et al. "Statin-naïve anti-HMGCR Antibody-mediated Necrotizing Myopathy in China." Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia, vol. 57, 2018, pp. 13-19.
Jiao Y, Cai S, Lin J, et al. Statin-naïve anti-HMGCR antibody-mediated necrotizing myopathy in China. J Clin Neurosci. 2018;57:13-19.
Jiao, Y., Cai, S., Lin, J., Zhu, W., Xi, J., Li, J., Yue, D., Zhang, T., Qiao, K., Wang, Y., Zhao, C., & Lu, J. (2018). Statin-naïve anti-HMGCR antibody-mediated necrotizing myopathy in China. Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia, 57, 13-19. https://doi.org/10.1016/j.jocn.2018.08.010
Jiao Y, et al. Statin-naïve anti-HMGCR Antibody-mediated Necrotizing Myopathy in China. J Clin Neurosci. 2018;57:13-19. PubMed PMID: 30205933.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Statin-naïve anti-HMGCR antibody-mediated necrotizing myopathy in China. AU - Jiao,Yuqiong, AU - Cai,Shuang, AU - Lin,Jie, AU - Zhu,Wenhua, AU - Xi,Jianying, AU - Li,Jin, AU - Yue,Dongyue, AU - Zhang,Tiansong, AU - Qiao,Kai, AU - Wang,Yin, AU - Zhao,Chongbo, AU - Lu,Jiahong, Y1 - 2018/09/08/ PY - 2018/01/28/received PY - 2018/06/24/revised PY - 2018/08/08/accepted PY - 2018/9/13/pubmed PY - 2018/12/12/medline PY - 2018/9/13/entrez KW - 3-Hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) KW - Immune-mediated necrotizing myopathy KW - Statin-naïve SP - 13 EP - 19 JF - Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia JO - J Clin Neurosci VL - 57 N2 - This study aimed to clarify the phenotypes and therapeutic responses of statin-naïve anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibody-mediated necrotizing myopathy. Anti-HMGCR antibodies were tested with ELISA methodology in the sera sample of 98 patients meeting the idiopathic inflammatory myopathy criteria and with negative anti-signal recognition particle (SRP) antibody. Twenty-one statin-naïve patients with anti-HMGCR antibody were detected (21.4%), with onset age from 6 to 67 years old. Proximal weakness and neck flexion weakness was the core neurological feature. The average maximal creatine kinase (CK) level was 7968.6 ± 4408.7U/L. Muscle MR imaging showed edema (88.2%), moderate or severe fatty replacement (70.6%) and muscle atrophy (88.2%) in lower limbs. Fatty replacement was significantly more prominent in the medial and posterior musculature than the anterior musculature (p = 0.0013). Seven (33.3%) patients were treated with mono-glucocorticoid, and thirteen (61.9%) patients needed adjuvant immunosuppressant. Eight (38.1%) patients experienced symptom relapse. The early-onset patients (<50 years old) were found with higher CK levels, shorter duration course, poorer response to adjuvant immunosuppressant and more recurrent weakness than the late-onset patients (≥50 years old). As a conclusion, Statin-naïve anti-HMGCR antibody-mediated necrotizing myopathy may not be rare. Compared with late-onset statin-naïve patients with anti-HMGCR antibody-mediated necrotizing myopathy, early-onset patients presented severer clinical features and worse therapeutic responses. SN - 1532-2653 UR - https://www.unboundmedicine.com/medline/citation/30205933/Statin_naïve_anti_HMGCR_antibody_mediated_necrotizing_myopathy_in_China_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0967-5868(18)30181-4 DB - PRIME DP - Unbound Medicine ER -