Muscular and extramuscular features of myositis patients with anti-U1-RNP autoantibodies

Maria Casal-Dominguez, Iago Pinal-Fernandez, Andrea Markl Corse, Julie Paik, Jemima Albayda, Livia A Casciola Rosen, Cheilonda Johnson, Sonye Danoff, Lisa Christopher-Stine, Eleni Tiniakou, Andrew L. Mammen

Research output: Contribution to journalArticle

Abstract

OBJECTIVE: To define the clinical phenotype of patients with myositis with anti-U1-ribonucleoprotein (RNP) autoantibodies. METHODS: 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). RESULTS: 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 p < 0.01). DM-specific skin features developed in 60% of anti-U1-RNP-positive patients. Interstitial lung disease (ILD) occurred in 45% of anti-U1-RNP-positive patients; fewer patients with DM (13%) and IMNM (6%) and more patients with AS (80%) developed ILD (all p < 0.01). Glomerulonephritis and pericarditis occurred in 25% and 40% of anti-U1-RNP-positive patients, respectively, but rarely in the other groups; these features occurred only in those with coexisting anti-Ro52 autoantibodies. No anti-U1-RNP patient had cancer-associated myositis or died during the study period. CONCLUSIONS: 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.

Original languageEnglish (US)
Pages (from-to)e1416-e1426
JournalNeurology
Volume92
Issue number13
DOIs
StatePublished - Mar 26 2019

Fingerprint

Myositis
Ribonucleoproteins
Autoantibodies
Dermatomyositis
Muscular Diseases
Interstitial Lung Diseases
Pericarditis
Muscle Weakness
Glomerulonephritis
Arthritis
Biopsy

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Muscular and extramuscular features of myositis patients with anti-U1-RNP autoantibodies. / Casal-Dominguez, Maria; Pinal-Fernandez, Iago; Corse, Andrea Markl; Paik, Julie; Albayda, Jemima; Casciola Rosen, Livia A; Johnson, Cheilonda; Danoff, Sonye; Christopher-Stine, Lisa; Tiniakou, Eleni; Mammen, Andrew L.

In: Neurology, Vol. 92, No. 13, 26.03.2019, p. e1416-e1426.

Research output: Contribution to journalArticle

@article{b7554a2ad27d4a4289d32460a56d6654,
title = "Muscular and extramuscular features of myositis patients with anti-U1-RNP autoantibodies",
abstract = "OBJECTIVE: To define the clinical phenotype of patients with myositis with anti-U1-ribonucleoprotein (RNP) autoantibodies. METHODS: 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). RESULTS: 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 p < 0.01). DM-specific skin features developed in 60{\%} of anti-U1-RNP-positive patients. Interstitial lung disease (ILD) occurred in 45{\%} of anti-U1-RNP-positive patients; fewer patients with DM (13{\%}) and IMNM (6{\%}) and more patients with AS (80{\%}) developed ILD (all p < 0.01). Glomerulonephritis and pericarditis occurred in 25{\%} and 40{\%} of anti-U1-RNP-positive patients, respectively, but rarely in the other groups; these features occurred only in those with coexisting anti-Ro52 autoantibodies. No anti-U1-RNP patient had cancer-associated myositis or died during the study period. CONCLUSIONS: 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.",
author = "Maria Casal-Dominguez and Iago Pinal-Fernandez and Corse, {Andrea Markl} and Julie Paik and Jemima Albayda and {Casciola Rosen}, {Livia A} and Cheilonda Johnson and Sonye Danoff and Lisa Christopher-Stine and Eleni Tiniakou and Mammen, {Andrew L.}",
year = "2019",
month = "3",
day = "26",
doi = "10.1212/WNL.0000000000007188",
language = "English (US)",
volume = "92",
pages = "e1416--e1426",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "13",

}

TY - JOUR

T1 - Muscular and extramuscular features of myositis patients with anti-U1-RNP autoantibodies

AU - Casal-Dominguez, Maria

AU - Pinal-Fernandez, Iago

AU - Corse, Andrea Markl

AU - Paik, Julie

AU - Albayda, Jemima

AU - Casciola Rosen, Livia A

AU - Johnson, Cheilonda

AU - Danoff, Sonye

AU - Christopher-Stine, Lisa

AU - Tiniakou, Eleni

AU - Mammen, Andrew L.

PY - 2019/3/26

Y1 - 2019/3/26

N2 - OBJECTIVE: To define the clinical phenotype of patients with myositis with anti-U1-ribonucleoprotein (RNP) autoantibodies. METHODS: 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). RESULTS: 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 p < 0.01). DM-specific skin features developed in 60% of anti-U1-RNP-positive patients. Interstitial lung disease (ILD) occurred in 45% of anti-U1-RNP-positive patients; fewer patients with DM (13%) and IMNM (6%) and more patients with AS (80%) developed ILD (all p < 0.01). Glomerulonephritis and pericarditis occurred in 25% and 40% of anti-U1-RNP-positive patients, respectively, but rarely in the other groups; these features occurred only in those with coexisting anti-Ro52 autoantibodies. No anti-U1-RNP patient had cancer-associated myositis or died during the study period. CONCLUSIONS: 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.

AB - OBJECTIVE: To define the clinical phenotype of patients with myositis with anti-U1-ribonucleoprotein (RNP) autoantibodies. METHODS: 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). RESULTS: 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 p < 0.01). DM-specific skin features developed in 60% of anti-U1-RNP-positive patients. Interstitial lung disease (ILD) occurred in 45% of anti-U1-RNP-positive patients; fewer patients with DM (13%) and IMNM (6%) and more patients with AS (80%) developed ILD (all p < 0.01). Glomerulonephritis and pericarditis occurred in 25% and 40% of anti-U1-RNP-positive patients, respectively, but rarely in the other groups; these features occurred only in those with coexisting anti-Ro52 autoantibodies. No anti-U1-RNP patient had cancer-associated myositis or died during the study period. CONCLUSIONS: 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.

UR - http://www.scopus.com/inward/record.url?scp=85063712264&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85063712264&partnerID=8YFLogxK

U2 - 10.1212/WNL.0000000000007188

DO - 10.1212/WNL.0000000000007188

M3 - Article

C2 - 30824556

AN - SCOPUS:85063712264

VL - 92

SP - e1416-e1426

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 13

ER -