Juvenile idiopathic inflammatory myopathies with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies in a Chinese cohort.

Our study indicates that anti-HMGCR antibodies may not be rare in Chinese JIIM. These anti-HMGCR-positive JIIMs were characterized by acute onset, substantially elevated creatine kinase level, and skin lesions without perifascicular changes in muscle pathology. The treatment outcome is generally favorable with the combination of steroid and immunosuppressant.