Dysregulation of the type I interferon system in adult‐onset clinically amyopathic dermatomyositis has a potential contribution to the development of interstitial lung disease

作者：Wei Sun, Yutong Sun, Hongjun Lin, Bing Yan, Guixiu Shi · 发表于：British Journal of Dermatology · 年份：2012 · DOI：10.1111/j.1365-2133.2012.11145.x · 被引用次数：25 · 研究领域：Inflammatory Myopathies and Dermatomyositis、Systemic Sclerosis and Related Diseases、Liver Diseases and Immunity

BACKGROUND: It has been speculated that viral infection might be one of the potential aetiologies for adult-onset clinically amyopathic dermatomyositis (CADM). The molecular pathogenesis remains largely unknown. OBJECTIVES: To explore whether dysregulation of the type I interferon (IFN) system is involved in the pathogenesis of CADM. METHODS: We studied 16 patients with CADM and compared them with healthy control subjects (n = 20) and patients with classic dermatomyositis (DM, n = 16) and polymyositis (PM, n = 16). Expressions of mRNA for serial toll-like receptor genes (TLR2, TLR3, TLR4, TLR7, TLR8 and TLR9) and type I IFN-regulated genes (IRF7, ISG15 and MxA) in peripheral blood leucocytes (PBL) were detected by real-time polymerase chain reaction analysis. The level of IFN-α in blood was tested by enzyme-linked immunosorbent assay. RESULTS: The mRNA expressions of TLR7, TLR9 and IRF7 were greatly elevated in the PBL from patients with CADM compared with controls. Upregulation of the ISG15 and MxA genes was detected in the PBL from patients with CADM, as well as from patients with classic DM. Among the four study groups, the overproduction of IFN-α in blood was most significant in the CADM group. Especially, IFN-α level was obviously high in the clinical interstitial lung disease (ILD) subgroup of patients with CADM. Positive correlations were found between IFN-α concentration and other unfavourable prognostic factors of CADM-associated ILD. CONCLUSIONS: Our data suggest th...