Presentation
The main presenting feature of NAM is subacute severe symmetrical proximal myopathy, with a markedly elevated creatine kinase (CK) level. [orpha.net]
The clinical presentation is highly variable, often similar to the other inflammatory myopathies. The most common finding is nevertheless the severe form with rhabdomyolysis. [pubmed.ncbi.nlm.nih.gov]
729-730 DOI: 10.17235/reed.2020.7324/2020 Eduardo Valdivielso Cortázar, Manuel Delgado Blanco, Pedro Alonso Aguirre, Abstract We present the case of an 87-year-old female who presented with upper dysphagia to solids, weight loss of 5 kg and weakness in [reed.es]
Treatment
Management and treatment Treatment of the underlying cause, if identified, is essential (statin discontinuation, or malignancy). [orpha.net]
Abatacept in the treatment of adult dermatomyositis and polymyositis: a randomised, phase IIb treatment delayed-start trial. Ann Rheum Dis 2018; 77 (1): 55-62. [ Links ] 67. Furlan A, Botsios C, Ruffatti A, Todesco S, Punzi L. [scielo.cl]
It includes consensus treatment recommendations for the different IMNM subsets. Neuromuscul Disord. 2018 Jan; 28(1):87-99. IV. Senecal JL, Raynauld JP, Troyanov Y. A new classification of adult autoimmune myositis (editorial). [ojs.reumatologia.org.ar]
Treatment is based on corticosteroid therapy, immunosuppressive drugs or intravenous immunoglobulins. Response is variable, depending on the clinical form. [pubmed.ncbi.nlm.nih.gov]
Treatment with oral steroids and methotrexate was started with a good subsequent evolution. New comment Comments No comments for this article References 1. Pinal-Fernández I, Casal Domínguez M, Mammen AL. Inmune-Mediated Necrotizing Myopathy. [reed.es]
Etiology
Etiology The disease is thought to be related to an immune response possibly triggered by drug therapy (statins), connective tissue diseases, or cancer. [orpha.net]
Epidemiology
Summary Epidemiology The prevalence and annual incidence of NAM are not known but the disorder is very rare. About 300 cases have been reported to date. Clinical description Age of onset ranges from 30 to 70 years of age in reported cases. [orpha.net]
Epidemiology of adult idiopathic inflammatory myopathies in a U.S. Managed care plan. Muscle Nerve 2012; 45 (5):676-83. [ Links ] 4. Needham M, Corbett A, Day T, Christiansen F, Fabian V, Mastaglia F. [scielo.cl]