TY  - BOOK
AU  - Huapaya, Julio
TI  - More prominent muscle involvement in patients with dermatomyositis with anti-Mi2 autoantibodies
SN  - 0028-3878
PY  - 2019///
KW  - *Autoantibodies/im [Immunology]
KW  - *Calcinosis/ep [Epidemiology]
KW  - *Dermatomyositis/im [Immunology]
KW  - *Fever/ep [Epidemiology]
KW  - *Lung Diseases, Interstitial/ep [Epidemiology]
KW  - *Mi-2 Nucleosome Remodeling and Deacetylase Complex/im [Immunology]
KW  - *Muscle Weakness/ep [Epidemiology]
KW  - Adult
KW  - Aged
KW  - Calcinosis/pp [Physiopathology]
KW  - Case-Control Studies
KW  - Cohort Studies
KW  - Creatine Kinase/bl [Blood]
KW  - Dermatomyositis/bl [Blood]
KW  - Dermatomyositis/ep [Epidemiology]
KW  - Dermatomyositis/pp [Physiopathology]
KW  - Female
KW  - Humans
KW  - Longitudinal Studies
KW  - Lung Diseases, Interstitial/pp [Physiopathology]
KW  - Male
KW  - Middle Aged
KW  - Muscle Weakness/pp [Physiopathology]
KW  - Myositis/im [Immunology]
KW  - Myositis/pp [Physiopathology]
KW  - Necrosis
KW  - Phenotype
KW  - Prevalence
KW  - Severity of Illness Index
KW  - MedStar Washington Hospital Center
KW  - Medicine/Internal Medicine
KW  - Journal Article
N1  - Available online from MWHC library: 1995 - present, Available in print through MWHC library: 1999 - 2006
N2  - 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; 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; OBJECTIVE: To define the clinical phenotype of dermatomyositis (DM) with anti-Mi2 autoantibodies; 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
UR  - https://dx.doi.org/10.1212/WNL.0000000000008443
ER  -