Abstract
Background
The aim of this study was to compare the heterogeneity of interstitial lung disease
(ILD) in patients with polymyositis and dermatomyositis (PM/DM) according to serological
type.
Methods
A total of 182 patients with PM/DM-ILD were observed retrospectively. Antiaminoacyl-tRNA
synthetase (ARS) and antimelanoma differentiation-associated gene5 (MDA5) antibodies
were screened using immunoblotting approach. The patients with ILD were divided into
3 groups: MDA5 (with anti-MDA5 antibody), ARS (with anti-ARS antibody) and MSN (without
anti-MDA5 or anti-ARS antibody) group. Pulmonary features, treatment responses and
prognoses were compared among the groups.
Results
A higher percentage of rapidly progressive ILD (RP-ILD) occurrences (55.8% versus
25% versus 16.9%, P < 0.001) was observed in the MDA5 group compared to ARS and MSN groups. The MSN group
experienced lower dyspnea (48.2% versus 79% versus 71.4%, P = 0.001) and fever (18.1% versus 39.5% versus 37.5%, P = 0.01) frequencies compared to MDA5 and ARS groups. Response to 6-month treatment
among 95 patients showed highest deterioration ratio (70%, P = 0.001) of ILD in the MDA5 group. Additionally, the highest frequency of ILD improvement
(60%, P = 0.04) was observed in the ARS group. During the observation period, 24 patients
died of respiratory failure. The 5-year survival rates were significantly lower in
MDA5 group (50.2%) compared to ARS group (97.7%) or the MSN group (91.4%) (P < 0.001).
Conclusions
MDA5-ILD was associated with severe pulmonary manifestations, poor response to treatment
and aggravated prognosis. The ARS-ILD group had favorable treatment response and prognosis.
MSN-ILD patients had relatively worse treatment response and prognosis compared to
the ARS group, even though they expressed milder pulmonary manifestation.
Key Indexing Terms
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Article info
Publication history
Published online: August 22, 2017
Accepted:
July 31,
2017
Received in revised form:
July 26,
2017
Received:
March 29,
2017
Footnotes
☆The authors have no financial or other conflicts of interest to disclose.
☆☆This work was supported by the National Natural Science Foundation of China, China (91542121 and 81571603), the Capital Foundation of Medical Development, China (No. 2016-2-4063), and the Science and Technology Commission Foundation of Beijing (Z151100004015143).
Identification
Copyright
© 2017 Southern Society for Clinical Investigation. Published by All rights reserved.
