Abstract
Abstract Background Anti-HMGCR (hydroxy-3-methylglutaryl-CoA reductase) myopathy is a form of myopathy described in patients on statins and Immune-mediated necrotizing myopathy (IMNM) Case summary We present the case of a 44-year-old female, who presented with progressive, bilateral, proximal muscle weakness. She was diagnosed with anti-HMGCR myopathy and showed an improvement in weakness after Rituximab therapy. Conclusion Anti-HMGCR myopathy has a wide variety of clinical presentations and the diagnosis of the rare, chronic, LGMD-like phenotype is particularly challenging. A mild to moderate elevation of CK is seen and imaging shows asymmetric muscle involvement. Definitive diagnosis is made by demonstrating anti-HMGCR antibodies in the serum. Treatment is individualized and should be initiated immediately with immunosuppressive therapy. Current therapeutic regimens lack strong substantial evidence however, a multi-drug regime is usually required. Rituximab may be used in patients with refractory disease as it can help wean off IVIG and taper steroids.
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