Treatment and prognosis of inclusion body myositis
- Marc L Miller, MD
Marc L Miller, MD
- Clinical Assistant Professor of Medicine
- Tufts University School of Medicine
- Section Editors
- Ira N Targoff, MD
Ira N Targoff, MD
- Section Editor — Muscle Disease
- Professor of Medicine, Section of Rheumatology
- University of Oklahoma Health Sciences Center
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
Sporadic inclusion body myositis (IBM) is classified along with polymyositis and dermatomyositis as one of the idiopathic inflammatory myopathies. However, despite some histologic similarities, the clinicopathologic manifestations, treatment, and prognosis of IBM are clearly distinct from the other two disorders (table 1). (See "Clinical manifestations of dermatomyositis and polymyositis in adults" and "Initial treatment of dermatomyositis and polymyositis in adults" and "Treatment of recurrent and resistant dermatomyositis and polymyositis in adults".)
The treatment and prognosis of IBM will be reviewed here. The clinical manifestations and diagnosis are presented separately. (See "Clinical manifestations and diagnosis of inclusion body myositis".)
In contrast to other inflammatory myopathies such as dermatomyositis and polymyositis, inclusion body myositis (IBM) is relatively resistant to standard immunosuppressive therapy, with muscle strength responding minimally, if at all, to glucocorticoids and other agents. It has been estimated that, in order to demonstrate a significant effect from treatment for IBM in a placebo-controlled study, 200 subjects would need to be enrolled in a six-month study or 100 in a year-long trial . This estimate should be kept in mind when considering the data on efficacy of treatment presented below. (See 'Specific therapies' below.)
Approach to therapy — The infrequent improvement in muscle strength in IBM has led some to consider the goal of therapy to be prevention of further deterioration in strength rather than improvement in strength . One problem is that IBM is often diagnosed years after the onset of symptoms. There may already be significant muscle damage by this time, preventing improvement in strength even if the disease process can be arrested.
Another limitation is that ongoing inflammation may not be responsible for continued or progressive muscle weakness. As an example, serial muscle biopsies obtained before and after glucocorticoid treatment have demonstrated resolution of inflammation and a decrease in the number of muscle fibers invaded by inflammatory cells in patients with no clinical improvement . Furthermore, the number of vacuolated fibers and the extent of amyloid deposition increased over time. (See "Clinical manifestations and diagnosis of inclusion body myositis".)
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