Update on Biomarkers of Vasculopathy in Juvenile and Adult Myositis.

PURPOSE OF REVIEW: Although rare, idiopathic inflammatory myopathies (IIM) comprise a heterogeneous group of autoimmune conditions in adults and children. Increasingly, vasculopathy is recognised to be key in the underlying pathophysiology and plays a crucial role in some of the more challenging complications including calcinosis, gastrointestinal involvement and interstitial lung disease. The exciting prospect of development of biomarkers of vasculopathy would enable earlier detection and monitoring of these complications and possible prevention of their potentially devastating consequences. The purpose was to review the current literature on biomarkers of vasculopathy in IIM and offer insight as to the biomarkers most likely to have an impact on clinical care.

RECENT FINDINGS: Multiple candidate biomarkers have been studied including circulating endothelial cells (CEC), microparticles (MP), soluble adhesion markers (ICAM-1, ICAM-3, VCAM-1), selectin proteins (E-, L-, P-selectin), coagulation factors, angiogenic factors, cytokines (including (IL-6, IL-10, TNF-α, IL-18) and interferon (IFN)-related biomarkers (including IFNα, IFN-β, IFNγ, galectin-9, interferon signature and interferon-related chemokines (MCP-1, IP-10 and MIG). There is a growing body of evidence of the potential role of biomarkers in detecting and monitoring the vasculopathy in IIM, detecting disease activity and predicting disease flares and overall prognosis. Exciting progress has been made in the search for biomarkers of vasculopathy of IIM; however, none of the studies are validated and further research is required.