title: Respiratory failure as presenting symptom of necrotizing autoimmune myopathy with anti-melanoma differentiation-associated gene 5 antibodies.
abstract: Necrotizing autoimmune myopathy (NAM) is a rare variant of idiopathic inflammatory myopathies associated with various underlying conditions. We describe a 48-year-old woman with a history of breast cancer who presented with acute respiratory failure and progressive loss of strength in her limb muscles over a period of two months. Muscle biopsy was consistent with necrotizing myopathy. No signs of active tumour were found. Immunoblot was positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies. Based on the subacute onset and muscle biopsy a diagnosis of NAM was made. Treatment with immunosuppressive and immunomodulating therapy led to almost complete recovery. Respiratory insufficiency as a presenting symptom of NAM is unusual. Recognition of this manifestation is crucial as early and aggressive treatment can lead to substantial recovery.
authors.affiliation: Department of Neurology, Academic Medical Centre, Amsterdam. Electronic address: b.jaeger@amc.uva.nl.
authors.forename: Bregje
authors.initials: B
authors.lastname: Jaeger
authors.affiliation: Department of Neurology, Academic Medical Centre, Amsterdam.
authors.forename: Marianne
authors.initials: M
authors.lastname: de Visser
authors.affiliation: Department of Pathology, Academic Medical Centre, Amsterdam.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Aronica
authors.affiliation: Department of Neurology, Academic Medical Centre, Amsterdam.
authors.forename: Anneke J
authors.initials: AJ
authors.lastname: van der Kooi
mesh_terms: D000906:Antibodies; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D053487:DEAD-box RNA Helicases / Q000276:immunology; D005260:Female; D006801:Humans; D000072640:Interferon-Induced Helicase, IFIH1; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009220:Myositis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D009336:Necrosis; D012131:Respiratory Insufficiency / Q000150:complications*
chemical_list: D000906:Antibodies; C452815:IFIH1 protein, human; D053487:DEAD-box RNA Helicases; D000072640:Interferon-Induced Helicase, IFIH1
keywords: Autoimmune; Myositis specific antibodies; Necrotizing myopathy; Paraneoplastic myopathy; Respiratory insufficiency