Persistent hyperCKemia as the sole initial manifestation of an idiopathic inflammatory myopathy
DOI:
https://doi.org/10.32818/reccmi.a11n2a12Keywords:
idiopathic inflammatory myopathies, hyperCKaemia, dermatomyositis, muscle biopsy, electromyographyAbstract
Idiopathic inflammatory myopathies may present atypically, without objective muscle weakness or detectable
myositis-related autoantibodies, making early diagnosis challenging. We report a case in which persistent hyperCKaemia led to the diagnosis through a structured diagnostic approach.A 48-year-old woman presented with
longstanding fatigue, myalgia and persistent creatine kinase levels above 2,500 U/L, despite preserved muscle
strength. Endocrine, infectious, toxic and neoplastic causes were excluded. Conventional immunological testing
and an extended myositis-specific autoantibody panel were negative. Electromyography demonstrated findings
consistent with inflammatory myopathy, while muscle biopsy confirmed idiopathic inflammatory myopathy with
histopathological features compatible with dermatomyositis. Treatment with high-dose glucocorticoids followed by azathioprine after methotrexate intolerance resulted in marked clinical improvement and near-normalisation of serum creatine kinase.
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Allenbach Y, Benveniste O. Inflammatory Myopathies. N Engl J Med. 2026; 394(19): 1925-38. doi: https://doi.org/10.1056/nejmra2415426 (último acceso ago. 2026). DOI: https://doi.org/10.1056/NEJMra2415426
Oldroyd AGS, Callen JP, Chinoy H, et al. International guideline for idiopathic inflammatory myopathy-associated cancer screening: an IMACS initiative. Nat Rev Rheumatol. 2023;19: 805-17. doi: https://doi.org/10.1038/s41584-023-01045-w (último acceso ago. 2026). DOI: https://doi.org/10.1038/s41584-023-01045-w
Selva-O'Callaghan A, Trallero-Araguás E, Gil-Vila A. Miopatía inflamatoria idiopática. Med Clin (Barc). 2025; 164: 429-36. doi: https://doi.org/10.1016/j.medcli.2024.12.008 (último acceso ago. 2026). DOI: https://doi.org/10.1016/j.medcli.2024.12.008
Selva-O'Callaghan A, Trallero-Araguás E, Gil-Vila A, et al. Treatment algorithms for inflammatory myopathies in adults: from Guidelines to Clinical Practice. Curr Treat Options Rheumatol. 2025; 11:21. doi: https://doi.org/10.1007/s40674-025-00239-5 (último acceso ago. 2026). DOI: https://doi.org/10.1007/s40674-025-00239-5
Raaphorst J, van der Kooi AJ, Mecoli CA, et al. Advances in the classification and management of idiopathic inflammatory myopathies. Lancet Neurol. 2025; 24: 776-88. doi: https://doi.org/10.1016/s1474-4422(25)00233-9 (último acceso ago. 2026). DOI: https://doi.org/10.1016/S1474-4422(25)00233-9
Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med. 2016; 280: 8-23. doi: https://doi.org/10.1111/joim.12451 (último acceso ago. 2026). DOI: https://doi.org/10.1111/joim.12451
Jiang W, Shi J, Yang H, Tian X, Yang H, Chen Q, et al. Long-term outcomes and prognostic factors in patients with idiopathic inflammatory myopathies based on myositis-specific autoantibodies. Arthritis Care Res (Hoboken). 2023; 75: 1175-82. doi: https://doi.org/10.1002/acr.24993 (último acceso ago. 2026). DOI: https://doi.org/10.1002/acr.24993
Campanilho-Marques R, Fonseca JE, Machado PM. Treatment of idiopathic inflammatory myopathies. Joint Bone Spine. 2025; 92: 105932. doi: https://doi.org/10.1016/j.jbspin.2025.105932 (último acceso ago. 2026). DOI: https://doi.org/10.1016/j.jbspin.2025.105932
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Copyright (c) 2026 Marina Martínez-Camerano, Carmen Vicente-Langarita, Emilio Ignacio Abecia-Martínez, Juan Calavera-Ambrosi, Javier Moreno-Díaz
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