Abstract
Background. The epidermal growth factors amphiregulin (AREG) and heparin-binding epidermal growth factor (HB-EGF) are implicated in the pathogenesis of several autoimmune diseases, but their clinical and pathological roles in idiopathic inflammatory myopathy (IIM) are unclear. Methods. Serum AREG and HB-EGF levels were measured by ELISA in patients with IIM (n = 37), systemic sclerosis (n = 17), and rheumatoid arthritis (n = 10), and for seven age- and sex-matched healthy controls (HCs). Associations between serum AREG or HB-EGF levels and the clinical parameters were analyzed. Results. Serum AREG levels in IIM patients were significantly elevated compared to those in HCs (median, 20.7 and 10.7 pg/mL, respectively; p = 0.025). In particular, serum AREG levels in IIM patients with interstitial lung disease (ILD) were higher than those of HCs (22.4 pg/mL, p = 0.027). The disease duration in patients with elevated serum AREG levels was significantly shorter compared to those who had normal serum AREG levels (7 and 21 months, respectively; p = 0.0012). Serum HB-EGF levels were significantly increased in IIM patients with elevated CK levels (136.2 pg/mL; p = 0.020) and patients with anti-Mi-2 antibody (183.7 pg/mL; p = 0.045) compared to those in HCs (74.9 pg/mL). Conclusion. These results suggested that AREG could be a promising biomarker associated with early-phase IIM-related ILD, and that HB-EGF expression was associated with muscle injury and regeneration in IIM.
Highlights
The pathogenesis of idiopathic inflammatory myositis (IIM) is based on dysregulation of the immune system [1]
Results of the present study suggested that the association with clinical parameters of idiopathic inflammatory myopathy (IIM) clearly differed between AREG and heparin-binding epidermal growth factor (HB-epidermal growth factor (EGF))
We considered that serum AREG and HB-EGF levels are related to different inflammatory processes and locations in IIM and represent different aspects of IIM pathophysiology
Summary
The pathogenesis of idiopathic inflammatory myositis (IIM) is based on dysregulation of the immune system [1]. Systemic involvements are common in IIM and are closely associated with the prognosis. In IIM can be resistant to immunosuppressive therapies and results in poor prognosis [2]. Several processes are associated with ILD progression in IIM. Macrophage activation assessed using soluble CD163 as a biomarker was reported to be important in IIM-related ILD [4]. The precise pathogenic mechanisms of IIM-related ILD remain unclear, and biomarkers capable of predicting disease activities and prognosis are required. The epidermal growth factors amphiregulin (AREG) and heparin-binding epidermal growth factor (HB-EGF) are implicated in the pathogenesis of several autoimmune diseases, but their clinical and pathological roles in idiopathic inflammatory myopathy (IIM) are unclear
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