Study of Arimoclomol in Inclusion Body Myositis (IBM)

Inclusion Criteria: * Meet any of the European Neuromuscular Centre Inclusion Body Myositis research diagnostic criteria 2011 categories for IBM. * Demonstrate being able to arise from a chair without support from another person or device. * Able to ambulate at least 20 ft/6 meters with or without assistive device. Once arisen from the chair, participant may use any walking device, i.e. walker/frame, can, crutches, or braces. They cannot be supported by another person and cannot use furniture or wall for support. * Age at onset of weakness \>45 years. * Body weight of \>= 40 kg. * Able to give informed consent. Exclusion Criteria: * History of any of the following excludes subject participation in the study: chronic infection particularly HIV or Hepatitis B or C; cancer other than basal cell cancer less than five years prior; or other chronic serious medical illnesses. * Presence of any of the following on routine blood screening: White blood cells (WBC) \<3000; platelets \< 100,000; hematocrit \<30%; blood urea nitrogen (BUN) \>30 mg/dL; creatinine \>1.5 x upper limit of normal; symptomatic liver disease with serum albumin \<3 g/dL. * History of most recent creatine kinase \>15x the upper limit of normal without any other explanation besides IBM. * History of non-compliance with other therapies. * Use of testosterone except for physiologic replacement doses in case of androgen deficiency. Participants must have documented proof of the androgen deficiency. * Coexistence of…