Inclusion Body Myositis: Autoimmune or Myodegenerative Disease?

Inclusion body myositis (IBM) is a slowly progressive muscle disease that usually affects patients >50 years of age.1 Whereas other types of myositis are characterized by symmetric proximal weakness that responds to immunosuppressive therapy,2 patients with IBM often have asymmetric distal weakness that progresses despite treatment with steroids or other immunomodulatory therapies. Muscle biopsies from patients with IBM include not only infiltrating inflammatory cells but also rimmed vacuoles and intracellular protein inclusions not observed in other forms of myositis. Given the coexistence of inflammatory and degenerative features on muscle biopsy, along with the poor response to treatment, there has been a longstanding debate about whether myodegeneration or autoimmunity is the driving pathologic process in IBM.

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