Cuando el sistema inmune ataca al músculo sin previo aviso: miopatía necrotizante inmunomediada por anticuerpos HMGCR, reporte de un caso.
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Abstract
Immune-mediated necrotizing myopathy associated with anti-HMGCR antibodies is a rare autoimmune disorder characterized by progressive proximal muscle weakness and markedly elevated muscle enzymes, often linked to prior statin exposure. We report the case of a 63-year-old woman with a history of dyslipidemia on long-term atorvastatin therapy who developed severe muscle weakness, cephaloparesis, dysphagia, and respiratory compromise requiring mechanical ventilation. Initial studies showed markedly elevated creatine kinase levels (>20,000 U/L), a negative myositis panel, and clinical features consistent with inflammatory myopathy. She was initially treated with corticosteroids, intravenous immunoglobulin, and cyclophosphamide without satisfactory response. Therapeutic plasma exchange was initiated, followed by rituximab, mycophenolate, and additional immunoglobulin, leading to clinical improvement. Anti-HMGCR antibodies were subsequently detected, confirming the diagnosis. This case highlights the critical role of early diagnosis and multidisciplinary management in a potentially life-threatening and often underrecognized condition, particularly in regions like Latin America where clinical reports remain scarce.Keywords:
Miositis , Miopatía Necrotizante , Anticuerpos anti-HMGCR , estatinasReferences
Mammen AL, Tiniakou E. Intravenous immune globulin for statin-triggered autoimmune myopathy. N Engl J Med. 2015;373(17):1680-2. Disponible en: https://www.nejm.org/doi/full/10.1056/NEJMc1506163
Rueda Carrasco L, Fuentes Caraballo AE. Miopatía necrotizante inmunomediada por estatinas. En: Sociedad Extremeña de Medicina Interna. VII Libro de Casos Clínicos. Badajoz: SEXMI; 2020. p. 50–53. Disponible en: https://www.sexmi.es/img/docmanager/VII-Libro-Casos-Clinicos.pdf#page=53
Khoo T, Chinoy H. Miopatía necrosante inmunomediada por anti-HMGCR: Abordando los problemas pendientes. Autoimmun Rev [Internet]. 2023;22(12):103468. Disponible en: http://dx.doi.org/10.1016/j.autrev.2023.103468
Mammen AL, Chung T, Christopher-Stine L, Rosen P, Rosen A, Doeringet K, al. Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy. Arthritis Rheum. 2011;63(3):713-21. Disponible en: https://pubmed.ncbi.nlm.nih.gov/21360500/
Pinal-Fernandez I, Parks C, Werner JL, Albayda J, Paik J, Danoff S, et al. Longitudinal course of disease in a large cohort of myositis patients with autoantibodies recognizing the signal recognition particle. Arthritis Care Res. 2017;69(2):263-70. DOI: 10.3233/JAD-170650
Mohassel P, Mammen AL. Anti-HMGCR myopathy. J Neuromuscul Dis. 2018;5(1):11-20. DOI: 10.3233/JND-170282.
Christopher-Stine L, Casciola-Rosen LA, Hong G, Chung H, Corse A, Mammen A. A novel autoantibody recognizing 200-kd and 100-kd proteins is associated with an immune-mediated necrotizing myopathy. Arthritis Rheum. 2010;62(9):2757-66. DOI: 10.1002/art.27572
Tiniakou E. Statin-Associated Autoimmune Myopathy: Current Perspectives. Ther Clin Risk Manag. 2020 May 27;16:483-492. doi: 10.2147/TCRM.S197941.
Nazir S, Lohani S, Tachamo N, Poudel D, Donatoet A. Anti-HMGCR myopathy: A systematic review of case reports and case series. Cureus. 2017;9(4):e1246. DOI: 10.1097/RHU.0000000000000497
Allenbach Y, Mammen AL, Benveniste O, Stenzel W. 224th ENMC International Workshop: Clinico-sero-pathological classification of immune-mediated necrotizing myopathies. Neuromuscul Disord. 2018;28(1):87-99. DOI: 10.1016/j.nmd.2017.09.016
Watanabe Y, Uruha A, Suzuki S, Nakahara J, Hamanaka K, Takayama K, et al. http://orcid.org/0000-0001-9452-112XIchizo Nishino2et al. Clinical features and prognosis in anti-SRP and anti-HMGCR necrotising myopathy. J Neurol Neurosurg Psychiatry. 2016;87(10):1038-44. https://doi.org/10.1136/jnnp-2016-313166
Mammen AL. Statin-associated autoimmune myopathy. N Engl J Med. 2016;374(7):664-9. Disponible en: https://www.nejm.org/doi/full/10.1056/NEJMra1515161
Tiniakou E, Pinal-Fernandez I, Lloyd TE, Albayda J, Paik J, Werner J et al. More severe disease and slower recovery in younger patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase-associated autoimmune myopathy. Rheumatology. 2017;56(5):787-94. https://doi.org/10.1093/rheumatology/kew470.
Kruse RL, Albayda J, Vozniak SO, Lawrence CE, Goel R, Lokhandwala PM, et al. Therapeutic plasma exchange for the treatment of refractory necrotizing autoimmune myopathy. J Clin Apher. 2022;37(4):340-348. doi: 10.1002/jca.21968.
