UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Treatment and prognosis of inclusion body myositis

Author
Marc L Miller, MD
Section Editors
Ira N Targoff, MD
Jeremy M Shefner, MD, PhD
Deputy Editor
Monica Ramirez Curtis, MD, MPH

INTRODUCTION

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".)

TREATMENT

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 [1]. 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 [2]. 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 [3]. Furthermore, the number of vacuolated fibers and the extent of amyloid deposition increased over time. (See "Clinical manifestations and diagnosis of inclusion body myositis".)

         

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Wed Jun 03 00:00:00 GMT 2015.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Griggs RC. The current status of treatment for inclusion-body myositis. Neurology 2006; 66:S30.
  2. Wortmann RL. The dilemma of treating patients with inclusion body myositis. J Rheumatol 1992; 19:1327.
  3. Barohn RJ, Amato AA, Sahenk Z, et al. Inclusion body myositis: explanation for poor response to immunosuppressive therapy. Neurology 1995; 45:1302.
  4. Danon MJ, Friedman M. Inclusion body myositis associated with progressive dysphagia: treatment with cricopharyngeal myotomy. Can J Neurol Sci 1989; 16:436.
  5. Lotz BP, Engel AG, Nishino H, et al. Inclusion body myositis. Observations in 40 patients. Brain 1989; 112 ( Pt 3):727.
  6. Sayers ME, Chou SM, Calabrese LH. Inclusion body myositis: analysis of 32 cases. J Rheumatol 1992; 19:1385.
  7. Leff RL, Miller FW, Hicks J, et al. The treatment of inclusion body myositis: a retrospective review and a randomized, prospective trial of immunosuppressive therapy. Medicine (Baltimore) 1993; 72:225.
  8. Joffe MM, Love LA, Leff RL, et al. Drug therapy of the idiopathic inflammatory myopathies: predictors of response to prednisone, azathioprine, and methotrexate and a comparison of their efficacy. Am J Med 1993; 94:379.
  9. Yood RA, Smith TW. Inclusion body myositis and systemic lupus erythematosus. J Rheumatol 1985; 12:568.
  10. Cohen MR, Sulaiman AR, Garancis JC, Wortmann RL. Clinical heterogeneity and treatment response in inclusion body myositis. Arthritis Rheum 1989; 32:734.
  11. Beyenburg S, Zierz S, Jerusalem F. Inclusion body myositis: clinical and histopathological features of 36 patients. Clin Investig 1993; 71:351.
  12. Badrising UA, Maat-Schieman ML, Ferrari MD, et al. Comparison of weakness progression in inclusion body myositis during treatment with methotrexate or placebo. Ann Neurol 2002; 51:369.
  13. Hopkinson ND, Hunt C, Powell RJ, Lowe J. Inclusion body myositis: an underdiagnosed condition? Ann Rheum Dis 1993; 52:147.
  14. Cherin P, Pelletier S, Teixeira A, et al. Intravenous immunoglobulin for dysphagia of inclusion body myositis. Neurology 2002; 58:326.
  15. Dalakas MC, Koffman B, Fujii M, et al. A controlled study of intravenous immunoglobulin combined with prednisone in the treatment of IBM. Neurology 2001; 56:323.
  16. Dalakas MC, Sonies B, Dambrosia J, et al. Treatment of inclusion-body myositis with IVIg: a double-blind, placebo-controlled study. Neurology 1997; 48:712.
  17. Walter MC, Lochmüller H, Toepfer M, et al. High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study. J Neurol 2000; 247:22.
  18. Soueidan SA, Dalakas MC. Treatment of inclusion-body myositis with high-dose intravenous immunoglobulin. Neurology 1993; 43:876.
  19. Amato AA, Barohn RJ, Jackson CE, et al. Inclusion body myositis: treatment with intravenous immunoglobulin. Neurology 1994; 44:1516.
  20. Amato AA, Sivakumar K, Goyal N, et al. Treatment of sporadic inclusion body myositis with bimagrumab. Neurology 2014; 83:2239.
  21. Lahouti AH, Amato AA, Christopher-Stine L. Inclusion body myositis: update. Curr Opin Rheumatol 2014; 26:690.
  22. Peng A, Koffman BM, Malley JD, Dalakas MC. Disease progression in sporadic inclusion body myositis: observations in 78 patients. Neurology 2000; 55:296.
  23. Benveniste O, Guiguet M, Freebody J, et al. Long-term observational study of sporadic inclusion body myositis. Brain 2011; 134:3176.
  24. Cox FM, Titulaer MJ, Sont JK, et al. A 12-year follow-up in sporadic inclusion body myositis: an end stage with major disabilities. Brain 2011; 134:3167.
  25. Voermans NC, Vaneker M, Hengstman GJ, et al. Primary respiratory failure in inclusion body myositis. Neurology 2004; 63:2191.