Highlights
- •Statin-naive anti-HMGCR cases.
- •Pattern of muscle MRI.
- •Early-onset cases are severer.
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.
Keywords
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Article info
Publication history
Published online: September 08, 2018
Accepted:
August 8,
2018
Received:
January 28,
2018
Identification
Copyright
© 2018 Elsevier Ltd. All rights reserved.
