MedStar Authors catalog › Details for: More prominent muscle involvement in patients with dermatomyositis with anti-Mi2 autoantibodies.
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More prominent muscle involvement in patients with dermatomyositis with anti-Mi2 autoantibodies.

by Huapaya, Julio.
Citation: Neurology. 93(19):e1768-e1777, 2019 Nov 05..Journal: Neurology.Published: 2019; ISSN: 0028-3878.Full author list: Albayda J; Casal-Dominguez M; Christopher-Stine L; Corse AM; Danoff SK; Hosono Y; Huang W; Huapaya J; Johnson C; Mammen AL; Mecoli CA; Paik JJ; Pak K; Pinal-Fernandez I; Tiniakou E.UI/PMID: 31594859.Subject(s): *Autoantibodies/im [Immunology] | *Calcinosis/ep [Epidemiology] | *Dermatomyositis/im [Immunology] | *Fever/ep [Epidemiology] | *Lung Diseases, Interstitial/ep [Epidemiology] | *Mi-2 Nucleosome Remodeling and Deacetylase Complex/im [Immunology] | *Muscle Weakness/ep [Epidemiology] | Adult | Aged | Calcinosis/pp [Physiopathology] | Case-Control Studies | Cohort Studies | Creatine Kinase/bl [Blood] | Dermatomyositis/bl [Blood] | Dermatomyositis/ep [Epidemiology] | Dermatomyositis/pp [Physiopathology] | Female | Humans | Longitudinal Studies | Lung Diseases, Interstitial/pp [Physiopathology] | Male | Middle Aged | Muscle Weakness/pp [Physiopathology] | Myositis/im [Immunology] | Myositis/pp [Physiopathology] | Necrosis | Phenotype | Prevalence | Severity of Illness IndexInstitution(s): MedStar Washington Hospital CenterDepartment(s): Medicine/Internal MedicineActivity type: Journal Article.Medline article type(s): Journal ArticleDigital Object Identifier: https://dx.doi.org/10.1212/WNL.0000000000008443 (Click here) Abbreviated citation: Neurology. 93(19):e1768-e1777, 2019 Nov 05.Local Holdings: Available online from MWHC library: 1995 - present, Available in print through MWHC library: 1999 - 2006.Abstract: CONCLUSIONS: Patients with anti-Mi2-positive DM have more severe muscle disease than patients with anti-Mi2-negative DM or patients with AS. Anti-Mi2 autoantibody levels correlate with disease severity and may normalize in patients who enter remission. Copyright (c) 2019 American Academy of Neurology.Abstract: METHODS: In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up in patients with anti-Mi2-positive DM were compared to patients with anti-Mi2-negative DM, antisynthetase syndrome (AS), and immune-mediated necrotizing myopathy (IMNM). Longitudinal anti-Mi2 autoantibody titers were assessed.Abstract: OBJECTIVE: To define the clinical phenotype of dermatomyositis (DM) with anti-Mi2 autoantibodies.Abstract: RESULTS: A total of 58 patients with anti-Mi2-positive DM, 143 patients with anti-Mi2-negative DM, 162 patients with AS, and 170 patients with IMNM were included. Among patients with anti-Mi2-positive DM, muscle weakness was present in 60% at disease onset and occurred in 98% during longitudinal follow-up; fewer patients with anti-Mi2-negative DM developed weakness (85%; p = 0.008). Patients with anti-Mi2-positive DM were weaker and had higher creatine kinase (CK) levels than patients with anti-Mi2-negative DM or patients with AS. Muscle biopsies from patients with anti-Mi2-positive DM had prominent necrosis. Anti-Mi2 autoantibody levels correlated with CK levels and strength (p < 0.001). With treatment, most patients with anti-Mi2-positive DM had improved strength and CK levels; among 10 with multiple serum samples collected over 4 or more years, anti-Mi2 autoantibody titers declined in all and normalized in 3, 2 of whom stopped immunosuppressant treatment and never relapsed. Patients with anti-Mi2-positive DM had less calcinosis (9% vs 28%; p = 0.003), interstitial lung disease (5% vs 16%; p = 0.04), and fever (7% vs 21%; p = 0.02) than did patients with anti-Mi2-negative DM.

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