Sporadic inclusion body myositis (IBM) is one of the most common acquired muscle disorders in adults over 50 years old [1,2]. Progressive disease is characterized by atrophy and fatty infiltration of muscle tissue, resulting in muscle weakness [3–5]. In early disease, the quadriceps, deep finger flexors and the pharyngeal muscles are most frequently affected, restricting functional ability and quality of life [6–8]. IBM is the result of a complex and only partially understood interplay between muscle inflammation, degeneration and impaired proteostasis which results in the accumulation of proteins in rimmed vacuoles [9].

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