Session Information
Date: Sunday, October 21, 2018
Title: Muscle Biology, Myositis and Myopathies Poster I: Clinical Features and Disease Course
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Autoimmune myositis (AIM) is a constellation of rare chronic disease with progressive muscle weakness. Several autoantibodies are found to be highly correlated with IIM (myositis-associated autoantibodies, MAAs) or distinct clinical features among IIM (myositis-specific autoantibodies, MSAs). In the current study, we evaluated autoantibody profiles using LIA and analyzed their association with clinical features among patients with autoimmune myositis in Korea.
Methods: Patients with idiopathic inflammatory myopathies were enrolled at seven tertiary care university medical centers. Autoantibodies were examined using EUROLINE assay (for 16 antigens including Mi-2alpha, Mi-2beta, TIF1gamma, MDA5, NXP2, SAE1, Ku, PM-Scl100, PM-Scl75, Jo-1, SRP, PL-7, PL-12, EJ, OJ, Ro-52). Data on history, physical findings, and laboratory investigations were obtained by retrospective medical record review.
Results: A total of ninety-three patients were enrolled. Seventy-two percent (n=67) was women, and mean age at the diagnosis was 50.7 +/- 13.5 year-old. Patients with PM, DM, ADM, and OM were 37 (39.8%), 48 (51.6%), 6 (6.5%), and 10 (10.8%), respectively. Anti-Ro52 antibody was the most common antibodies, and anti-TIF1gamma antibody was the most common MSA (Table 1). Positive anti-TIF1gamma antibody was significantly associated with the presence of malignancy and absence of interstitial lung disease (ILD). ILD was observed among 42 patients (45.2 %) and of those ILD, anti-MDA5 antibody was associated with bronchiolitis obliterans organizing pneumonia/cryptogenic organizing pneumonia (BOOP/COP) and anti-synthetase antibodies were associated with nonspecific interstitial pneumonia (NSIP, Table 2).
Conclusion: Myositis-specific autoantibodies were related with clinical manifestations. Especially, anti-MDA5 and anti-synthetase were associated with BOOP/COP and NSIP, respectively.
Table 1. Autoantibody profiles
|
Antigen |
N |
% (of total 93) |
|
Mi-2 alpha |
7 |
7.5 |
|
Mi-2 beta |
5 |
5.4 |
|
TIF1 gamma |
15 |
16.1 |
|
MDA5 |
11 |
11.8 |
|
NXP2 |
1 |
1.1 |
|
SAE1 |
2 |
2.2 |
|
Ku |
4 |
4.3 |
|
PM-Scl100 |
1 |
1.1 |
|
PM-Scl75 |
8 |
8.6 |
|
Jo-1 |
13 |
14 |
|
SRP |
12 |
12.9 |
|
PL-7 |
4 |
4.3 |
|
PL-12 |
1 |
1.1 |
|
EJ |
4 |
4.3 |
|
OJ |
6 |
6.5 |
|
Ro-52 |
37 |
49.3 |
Table 2. Clinical features and autoantibodies
|
Clinical features |
autoantibodies |
odds ratio |
95% confidence interval |
P |
|
Malignancy |
Anti-TIF1gamma |
43.4 |
7.7-245.5 |
<0.01 |
|
ILD |
Anti-TIF1gamma |
0.3 |
0.7-1.0 |
=<0.05 |
|
¡¡
|
Anti-synthetase antibodies*
|
7.6 |
2.5-22.9 |
<0.01 |
|
ILD-BOOP/COP |
Anti-MDA5 |
7 |
1.8-27.1 |
<0.01 |
|
ILD-NSIP |
Anti-synthetase antibodies*
|
3.5 |
1.3-9.8 |
<0.05 |
*anti-synthetase antibodies include antibodies against Jo-1, PL-7, PL-12, EJ, and OJ
To cite this abstract in AMA style:
Chang SH, Lee SW, Kang MI, Kwon M, Joung CI, Kang SW, Yoo IS, Shim SC, Yoo SJ, Choi IA, Kim JH, Hong SJ, Lee YA, Chung SW, Kim J. Autoantibody Profile and Clinical Characteristics in Patients with Idiopathic Inflammatory Myopathies [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/autoantibody-profile-and-clinical-characteristics-in-patients-with-idiopathic-inflammatory-myopathies/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/autoantibody-profile-and-clinical-characteristics-in-patients-with-idiopathic-inflammatory-myopathies/