• Diana Prieto-Peña, Javier G Ocejo-Vinyals, Joel Mazariegos-Cano, Ana L Pelayo-Negro, Sara Remuzgo-Martínez, Fernanda Genre, Alicia García-Dorta, Mónica Renuncio-García, Víctor M Martínez-Taboada, Carmen García-Ibarbia, Julio Sánchez-Martín, Marcos López-Hoyos, Ricardo Blanco, Miguel A González-Gay, and José L Hernández.
• Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain; Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Santander, Spain. Electronic address: diana.prieto.pena@gmail.com.
• Eur. J. Intern. Med. 2022 Jul 1; 101: 86-92.

ObjectiveTo characterize the demographic, genetic, clinical, and serological features of patients with anti-3‑hydroxy-3-methylglutaryl-CoA reductase (HMGCR) immune-mediated necrotizing myopathy (IMNM) in a region of northern Spain.MethodsStudy of all patients diagnosed with anti-HMGCR IMNM during a 5-year period at a reference hospital in northern Spain. Besides clinical and laboratory data, we analyzed the genetic influence of HLA genes and the rs4149056 (c.521T>C) single nucleotide polymorphism (SNP) in the SLCO1B1 gene.Results8 patients (5 women, 3 men) with a mean ± SD age of 64.9 ± 7.3 years, fulfilled the criteria for anti-HMGCR IMNM. The incidence rate was 0.6 per 100.000 person-years and the prevalence 3 per 100.000 population. All patients had been exposed to statins. All of them had predominant lower limb proximal and symmetric muscle weakness that was severe in 2 and had elevated serum CK levels with a median [IQR] of 4488 [2538-9194] IU/L. Serum 25‑hydroxy vitamin D levels were decreased in all patients in whom it was determined. The 3 patients with a previous diagnosis of hypothyroidism had abnormal levels of TSH at the time of diagnosis. All patients experienced improvement with different schemes of immunosuppressive therapy. Noteworthy, 7 of 8 patients carried the HLA-DRB1*11 allele. The frequency of the rs4149056 C allele in the SLCO1B1 gene (12.5%) was similar to that of the general population.ConclusionIn northern Spain, anti-HMGCR IMNM preferentially affects people over 50 years of age who are carriers of the HLA-DRB1*11 allele and take statins. Both low vitamin D levels and hypothyroidism may play a potential predisposing role in the development of this disease.Copyright © 2022. Published by Elsevier B.V.

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