Muscular and extramuscular features of myositis patients with anti-U1-RNP autoantibodies.
Adult
Black or African American
Age of Onset
Aged
Arthritis
/ etiology
Autoantibodies
/ immunology
Autoimmune Diseases
/ complications
Case-Control Studies
Cohort Studies
Dermatomyositis
/ ethnology
Female
Glomerulonephritis
/ etiology
Humans
Longitudinal Studies
Male
Middle Aged
Muscle Weakness
/ etiology
Muscle, Skeletal
/ pathology
Myositis
/ complications
Necrosis
Pericarditis
/ etiology
Ribonucleoprotein, U1 Small Nuclear
/ immunology
White People
Young Adult
Journal
Neurology
ISSN: 1526-632X
Titre abrégé: Neurology
Pays: United States
ID NLM: 0401060
Informations de publication
Date de publication:
26 03 2019
26 03 2019
Historique:
received:
21
08
2018
accepted:
16
11
2018
pubmed:
3
3
2019
medline:
18
12
2019
entrez:
3
3
2019
Statut:
ppublish
Résumé
To define the clinical phenotype of patients with myositis with anti-U1-ribonucleoprotein (RNP) autoantibodies. In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up in patients with anti-U1-RNP-positive myositis were compared to those with dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM), and the antisynthetase syndrome (AS). Twenty anti-U1-RNP-positive patients, 178 patients with DM, 135 patients with IMNM, and 132 patients with AS were included. Anti-U1-RNP-positive patients were younger (∼37 years) and more likely to be black (60%) than patients with AS, DM, or IMNM. Muscle weakness was a presenting feature in 15% of anti-U1-RNP-positive patients; 80% eventually developed weakness. Four of 7 anti-U1-RNP-positive patients had necrotizing muscle biopsies. Arthritis occurred in 60% of anti-U1-RNP-positive patients; this was increased compared to DM (18%) or IMNM (6%) (all Patients with anti-U1-RNP myositis typically present with proximal weakness and necrotizing muscle biopsies. Arthritis, dermatitis, and ILD are the most common extramuscular clinical features. Pericarditis and glomerulonephritis are uniquely found in patients with anti-U1-RNP-positive myositis.
Identifiants
pubmed: 30824556
pii: WNL.0000000000007188
doi: 10.1212/WNL.0000000000007188
pmc: PMC6453768
doi:
Substances chimiques
Autoantibodies
0
Ribonucleoprotein, U1 Small Nuclear
0
Types de publication
Journal Article
Research Support, N.I.H., Intramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e1416-e1426Informations de copyright
© 2019 American Academy of Neurology.
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