Inclusion body myositis and immunosenescence: current evidence and future perspectives.

Abstract

IBM remains an enigmatic and complex muscle disorder where a deeper understanding of disease pathomechanisms and the identification of potential genetic contributors represent an unmet need. The absence of effective treatments has spurred endeavours to reassess the interplay between degeneration, including autophagy, mitochondrial dysfunction and proteasomal dysregulation, and autoimmunity. IBM is unique among the other idiopathic inflammatory myopathies owing to its molecular signature involving highly differentiated cytotoxic T cells that evade immune regulation. This has led to a resurgence of interest in the development of immunomodulatory therapy. This review discusses the potential role of cellular immunosenescence in sustaining inflammation and/or fibrotic remodelling observed in IBM and appraises the rationale for some potential therapeutic approaches to mitigate disease progression.