Inclusion-Body Myositis in the Elderly: An Update

Sporadic inclusion-body myositis is a common inflammatory myopathy, which is often misdiagnosed. In contrast to other forms of myositis, no effective treatment is available. The disease leads to severe wasting of the quadriceps and long-finger flexors, so patients gradually lose ambulation and hand-grip strength. The pathology includes an intrafiber accumulation of aberrant molecules, such as β-amyloid, as well as an inflammatory cascade, with overexpression of key cytokines and chemokines, and the attack of muscle fibers by autoaggressive cytotoxic T cells. Recent data point to an early cell-stress response in muscle fibers and a unique interplay between inflammatory and degenerative pathomechanisms. Current efforts aim to improve methods for early diagnosis and design more effective targeted treatment strategies. This article will highlight recent advances in understanding the disease pathology, and how to identify promising candidate molecules for future clinical trials.