Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) related myositis is a form of immune-mediated necrotizing myopathy (IMNM). Anti-HMGCR autoantibodies target HMGCR, a glycoprotein linked to the endoplasmic reticulum implied in the cholesterol synthesis pathway, and exert a pathogenic effect on skeletal muscle cells. More than 60% of patients affected by HMGCR-related myositis shares statin-exposure in their medical history. Patients commonly experience CK levels elevation, myalgia, muscle weakness and soreness at variable extent, which manifest acutely or sub acutely with a progressively worsening course, in some cases mimicking limb-girdle muscular dystrophies (LGMD) phenotype and treatment is based on an immunosuppressive strategy. Here we present the peculiar case of a previously statins-exposed 72 y.o. asymptomatic man with persistent moderate hyperCKemia and high levels of anti-HMGCR, in which pharmacotherapy has not been initiated yet, while a wait-and-see approach has been adopted instead.
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http://dx.doi.org/10.36185/2532-1900-050 | DOI Listing |
JCEM Case Rep
December 2024
Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, MN 55905, USA.
The widespread use of statins for cardiovascular diseases has unveiled a new subset of inflammatory myopathy, immune-mediated necrotizing myopathy (IMNM). We describe below an unusual case of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) myopathy. A 64-year-old male individual with type 2 diabetes, hyperlipidemia, and coronary artery disease presented with progressive proximal muscle weakness and pain for 3 months.
View Article and Find Full Text PDFFront Immunol
November 2024
Department of Neurology, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.
Immune-mediated necrotizing myopathy (IMNM) with anti-HMGCR antibody positivity is characterized by proximal extremity weakness, increased creatine kinase, and extensive muscle edema. There is an urgent need to find more appropriate treatment options for anti-HMGCR IMNM patients who do not respond well to conventional therapy in the acute phase. With the advent of targeted biologics, new treatment options are available.
View Article and Find Full Text PDFCureus
October 2024
Internal Medicine, Unidade Local de Saúde da Região de Aveiro, Aveiro, PRT.
Statin-induced necrotizing autoimmune myopathy (SINAM) is a rare, disabling, and potentially life-threatening complication of statin use. Although not fully understood, a prevailing hypothesis proposes that statins induce molecular changes in 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR), leading to the formation of anti-HMGCR antibodies. The subsequent myofibre necrosis is expressed as progressive and persistent proximal muscle weakness and elevated creatine kinase (CK) levels.
View Article and Find Full Text PDFPediatr Rheumatol Online J
November 2024
Rheumatology Department, Centre National de Référence des Maladies Auto- Immunes Rares (CERAINOM), Brest University Hospital, Brest, France.
Front Immunol
November 2024
Department of Neurology, Peking University First Hospital, Beijing, China.
Objective: We aimed to explore the efficacy and safety of efgartigimod in patients with refractory immune-mediated necrotizing myopathy (IMNM).
Methods: This open-label pilot observational study included seven patients with refractory IMNM, all of whom received intravenous efgartigimod treatment. The clinical response was assessed after 4 weeks of efgartigimod treatment according to the 2016 American College of Rheumatology-European League Against Rheumatism response criteria for adult idiopathic inflammatory myopathy.
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