NLRP3 and inclusion body myositis: Disrupted protein homeostasis with the aggregation of autophagic vacuoles and misfolded protein aggregates is a canonical histopathological feature of IBM.59, 60 Last, males had more significantly altered mitophagy than females, and NLRP3 levels correlated with the severity of muscle weakness in males supporting a direct association between NLPR3 activation and loss of muscle strength in this disease.