Abstract
ObjectiveTo investigate the significance of high mobility group box 1 (HMGB1) levels in polymyositis (PM) and dermatomyositis (DM) patients with interstitial lung disease and whether HMGB1 levels could predict disease outcome.MethodsHMGB1 levels were measured in sera from 34 patients with PM/DM and from 34 healthy controls by ELISA.ResultsSignificantly higher serum levels of HMGB1 were found in patients with PM [12.75 ng/ml (4.34–25.07 ng/ml), p < 0.001] and DM [20.75 ng/ml (3.80–124.88 ng/ml), p < 0.001] than in healthy controls [5.64 ng/ml (2.71–8.71 ng/ml)]. Importantly, the average HMGB1 level in PM/DM patients with interstitial lung disease (ILD) was 25.84 ng/ml, which is significantly higher than that in PM/DM patients without ILD [12.68 ng/ml] (p < 0.05). A receiver operating characteristic (ROC) curve analysis revealed that the serum HMGB1 cutoff value that best discriminated PM/DM patients with ILD from those without ILD was 14.5ng/ml. The area under the curve was 0.87±0.05, and the 95% Confidence interval (CI) was 0.77–0.98. The diagnostic sensitivity and specificity of this serum HMGB1 cutoff level was 84.6% and 89% respectively. Patients with higher levels of HMGB1 expression had lower overall survival rates and disease-free survival rates, whereas patients with lower levels of HMGB1 expression had higher survival rates.ConclusionMultivariate analysis showed that HMGB1 expression is a prognostic indicator for patient survival. These data support the notion that HMGB1 overexpression is involved in PM/DM progression for patients with ILD and is relative to its poor clinical outcomes.
Highlights
Polymyositis (PM) and Dermatomyositis (DM) are heterogeneous complex autoimmune diseases that primarily target skeletal muscles and skin
A receiver operating characteristic (ROC) curve analysis revealed that the serum high mobility group box 1 (HMGB1) cutoff value that best discriminated PM/DM patients with interstitial lung disease (ILD) from those without ILD was 14.5ng/ml
These data support the notion that HMGB1 overexpression is involved in PM/DM progression for patients with ILD and is relative to its poor clinical outcomes
Summary
Polymyositis (PM) and Dermatomyositis (DM) are heterogeneous complex autoimmune diseases that primarily target skeletal muscles and skin. They are characterized by progressive systematic proximal muscle weakness and erythematous rash. PM/DM can present with multi-organ or systemic manifestations and a significant increase in the incidence of interstitial lung disease (ILD) [1, 2]. The etiopathogenesis of PM/DM remains unclear, the hallmark of the disease is lymphocytic infiltration of muscles, muscle inflammation, and chronic muscle dystrophy [3, 4]. The prevailing paradigm is that muscle lesions in patients with PM/ DM are populated with CD4+ T and CD8+ T lymphocytes and MHC-I, which orchestrate muscular damage [5,6,7,8,9,10].
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