Correlation of increased serum leucine-rich α2-glycoprotein levels with disease prognosis, progression, and activity of interstitial pneumonia in patients with dermatomyositis: A retrospective study.

Takaaki Ishida,Takuya Kotani,Minoru Fujimoto,Tohru Takeuchi,Shigeki Makino,Tetsuji Naka,Satoshi Serada,Masataka Kuwana

doi:10.1371/journal.pone.0234090

Takaaki Ishida, Takuya Kotani + Show 6 more

Open Access

https://doi.org/10.1371/journal.pone.0234090

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Abstract

ObjectiveTo investigate whether leucine-rich α2-glycoprotein (LRG) can be a biomarker for the disease activity, progression, and prognosis of interstitial pneumonia (IP) in patients with dermatomyositis (DM).MethodsCorrelations between the clinical findings and serum LRG levels were investigated in 46 patients with DM-IP (33 with acute/subacute IP [A/SIP] and 13 patients with chronic IP [CIP], including 10 fatal cases of IP).ResultsThe median serum LRG level of 18.4 (14.6–25.2) μg/mL in DM-IP patients was higher than that in healthy control subjects. The median levels of serum LRG at baseline and at 2 and 4 weeks after the initiation of treatment in the patients who died were significantly higher than those in the surviving patients (P = 0.026, 0.029, and 0.008, respectively). The median level of serum LRG in the DM-A/SIP patients was significantly higher than that in the DM-CIP patients (P = 0.0004), and that in the anti-MDA5-Ab-positive group was slightly higher than that in the anti-ARS-Ab-positive group. The serum LRG levels correlated significantly with the serum levels of LDH, C-reactive protein, ferritin, AaDO2, %DLco, and total ground-glass opacity score. The survival rate after 24 weeks in patients with an initial LRG level ≥ 17.6 μg/mL (survival rate: 40%) was significantly lower than that in patients with an initial LRG level < 17.6 μg/mL (100%) (P = 0.0009).ConclusionThe serum LRG level may be a promising marker of disease activity, progression, and prognosis in patients with DM-IP.

Highlights

Dermatomyositis (DM) is frequently complicated by interstitial pneumonia (IP), which is associated with increased morbidity and mortality [1, 2]
Laboratory markers are presented as the median
The percentage with acute/subacute IP (A/SIP) was significantly higher in the IP death group (100%) than in the survivor group (64%) (P = 0.042)

Summary

Introduction

Dermatomyositis (DM) is frequently complicated by interstitial pneumonia (IP), which is associated with increased morbidity and mortality [1, 2]. DM-IP is classified as being either acute/subacute IP (A/SIP) or chronic IP (CIP), and A/SIP type progresses within 3 months. Anti-aminoacyl tRNA synthetase (ARS) antibody (Ab) and anti-melanoma differentiationassociated gene (MDA) 5 Ab are autoantibodies associated with DM-IP. Patients with advanced disease and are positive for anti-ARS Ab undergo immunosuppressive therapy with corticosteroids, calcineurin inhibitor, and/or intravenous pulse cyclophosphamide (IVCY). Most patients respond well to immunosuppressive therapy, some are refractory or experience recurrence [3, 4]. Anti-MDA5 Ab-positive DM-IP patients are immediately placed on aggressive immunosuppressive therapy, about half of these patients are resistant to the therapy and will die from respiratory failure within a few months [5, 6]. When determining the appropriate treatment, it is clinically important to discover factors predictive of the progression and prognosis of DM-IP

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