Treatment strategies for inclusion body myositis

Abstract

Introduction: Inclusion body myositis (IBM) is the most common acquired myopathy in mid-aged patients, leading to progressive muscle weakness and atrophy. IBM differs from other forms of myositis by the presence of myodegenerative features and its resistance to immunosuppressive treatment. So far, no effective treatment has been identified.

Areas covered: In this review, the authors aim to provide an overview of past and current treatment studies in IBM. A range of clinical trials and case series have been conducted in IBM, including those with immunosuppressants, immunomodulators such as intravenous immunoglobulins (IVIg) and anti-degenerative molecules such as arimoclomol and lithium. Based on critical assessment of our current understanding of the complex disease pathology, we discuss potential treatment strategies for future clinical trials.

Expert opinion: Lack of understanding of the disease pathology hampers the identification of successful therapeutic agents. Improvement of the diagnostic criteria and identification of biomarkers are needed to allow for an early diagnosis and timely start of treatment, ideally before irreversible muscle damage has occurred. Promising treatment aims include the reduction of cell-stress, protein-aggregation and inflammation as well as improvement of regeneration. Until an effective treatment for IBM has been identified, probationary treatment for 6 months with IVIg can be justified.

Keywords::

• autophagy
• cell stress
• heat-shock protein
• inclusion body myositis
• inflammatory myopathy
• IL-1β
• myostatin
• protein aggregation
• regeneration
• β-amyloid

Notes

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