Management of inclusion body myositis
- Thomas E Lloyd, MD, PhD
Thomas E Lloyd, MD, PhD
- Codirector, Johns Hopkins Myositis Center
- Associate Professor of Neurology, Neuroscience, and Genetics
- Johns Hopkins 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, dermatomyositis, and autoimmune necrotizing myopathy 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 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".)
GOALS OF THERAPY
The primary goal of therapy in inclusion body myositis (IBM) is to optimize muscle strength and function. Given the slowly progressive and variable course of the disease, it can be quite challenging to determine if treatment leads to an objective improvement in or stabilization of muscle strength . It is well known that immunosuppressive medications will lower muscle enzyme levels in IBM patients despite continued progression of weakness, and also that creatine kinase (CK) levels decrease with muscle atrophy [2,3]. Therefore, CK levels cannot be used to monitor response to therapy in this disease. Based on the existing data, we only consider a trial of immunosuppressive medications in IBM patients with an atypical presentation or in patients with another autoimmune disease.
Almost all patients with any degree of limitation in activities of daily living will benefit from physical and occupational therapy evaluation. Patients with dysphagia should be evaluated by a speech therapist. Exercise is likely beneficial in all patients. In contrast to other inflammatory myopathies such as dermatomyositis and polymyositis, inclusion body myositis (IBM) is relatively resistant to standard immunomodulatory therapies [4,5]. However, there is limited evidence that some subgroups of patients may benefit from such therapy, in particular those with another systemic autoimmune disease such as Sjögren's syndrome or systemic lupus erythematosus [6-8]. We typically suggest a trial of immunosuppressive treatment when the diagnosis of IBM is uncertain, or in cases where there appears to be overlap with polymyositis as evidenced by early and prominent proximal weakness (ie, neck flexors, arm abductors, hip flexors). (See 'Immunosuppressive therapy in selected patients' below.)
Nonpharmacologic therapy — The optimal treatment for IBM is not known, and most interventions have demonstrated only limited benefit. Nonpharmacologic interventions such as physical therapy, occupational therapy, and/or speech therapy can be helpful in all patients to achieve the following:
- Griggs RC. The current status of treatment for inclusion-body myositis. Neurology 2006; 66:S30.
- 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.
- Barohn RJ, Amato AA, Sahenk Z, et al. Inclusion body myositis: explanation for poor response to immunosuppressive therapy. Neurology 1995; 45:1302.
- Needham M, Mastaglia FL. Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment. Clin Neurophysiol 2016; 127:1764.
- Saltychev M, Mikkelsson M, Laimi K. Medication of inclusion body myositis: a systematic review. Acta Neurol Scand 2015.
- Yood RA, Smith TW. Inclusion body myositis and systemic lupus erythematosus. J Rheumatol 1985; 12:568.
- Cohen MR, Sulaiman AR, Garancis JC, Wortmann RL. Clinical heterogeneity and treatment response in inclusion body myositis. Arthritis Rheum 1989; 32:734.
- Beyenburg S, Zierz S, Jerusalem F. Inclusion body myositis: clinical and histopathological features of 36 patients. Clin Investig 1993; 71:351.
- Johnson LG, Collier KE, Edwards DJ, et al. Improvement in aerobic capacity after an exercise program in sporadic inclusion body myositis. J Clin Neuromuscul Dis 2009; 10:178.
- Alexanderson H. Exercise in inflammatory myopathies, including inclusion body myositis. Curr Rheumatol Rep 2012; 14:244.
- Spector SA, Lemmer JT, Koffman BM, et al. Safety and efficacy of strength training in patients with sporadic inclusion body myositis. Muscle Nerve 1997; 20:1242.
- Arnardottir S, Alexanderson H, Lundberg IE, Borg K. Sporadic inclusion body myositis: pilot study on the effects of a home exercise program on muscle function, histopathology and inflammatory reaction. J Rehabil Med 2003; 35:31.
- Bernhardt K, Oh T, Kaufman K. Stance control orthosis trial in patients with inclusion body myositis. Prosthet Orthot Int 2011; 35:39.
- Eriksson M, Lindberg C. Hand function in 45 patients with sporadic inclusion body myositis. Occup Ther Int 2012; 19:108.
- Lloyd TE, Mammen AL, Amato AA, et al. Evaluation and construction of diagnostic criteria for inclusion body myositis. Neurology 2014; 83:426.
- Waclawik AJ, Rao VK. Effective treatment of severe finger flexion weakness in inclusion body myositis using tendon transfers. J Clin Neuromuscul Dis 2002; 4:31.
- Cox FM, Verschuuren JJ, Verbist BM, et al. Detecting dysphagia in inclusion body myositis. J Neurol 2009; 256:2009.
- Jones K, Pitceathly RD, Rose MR, et al. Interventions for dysphagia in long-term, progressive muscle disease. Cochrane Database Syst Rev 2016; 2:CD004303.
- Murata KY, Kouda K, Tajima F, Kondo T. Balloon dilation in sporadic inclusion body myositis patients with Dysphagia. Clin Med Insights Case Rep 2013; 6:1.
- Darrow DH, Hoffman HT, Barnes GJ, Wiley CA. Management of dysphagia in inclusion body myositis. Arch Otolaryngol Head Neck Surg 1992; 118:313.
- Oh TH, Brumfield KA, Hoskin TL, et al. Dysphagia in inflammatory myopathy: clinical characteristics, treatment strategies, and outcome in 62 patients. Mayo Clin Proc 2007; 82:441.
- Kley RA, Vorgerd M, Tarnopolsky MA. Creatine for treating muscle disorders. Cochrane Database Syst Rev 2007; :CD004760.
- Chhibber S, Amato AA. Clinical Evaluation and Management of Inflammatory Myopathies. Semin Neurol 2015; 35:347.
- Dalakas MC. Inflammatory muscle diseases. N Engl J Med 2015; 372:1734.
- Lotz BP, Engel AG, Nishino H, et al. Inclusion body myositis. Observations in 40 patients. Brain 1989; 112 ( Pt 3):727.
- Sayers ME, Chou SM, Calabrese LH. Inclusion body myositis: analysis of 32 cases. J Rheumatol 1992; 19:1385.
- 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.
- Benveniste O, Guiguet M, Freebody J, et al. Long-term observational study of sporadic inclusion body myositis. Brain 2011; 134:3176.
- 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.
- Rutkove SB, Parker RA, Nardin RA, et al. A pilot randomized trial of oxandrolone in inclusion body myositis. Neurology 2002; 58:1081.
- Dobloug C, Walle-Hansen R, Gran JT, Molberg Ø. Long-term follow-up of sporadic inclusion body myositis treated with intravenous immunoglobulin: a retrospective study of 16 patients. Clin Exp Rheumatol 2012; 30:838.
- 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.
- 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.
- 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.
- Soueidan SA, Dalakas MC. Treatment of inclusion-body myositis with high-dose intravenous immunoglobulin. Neurology 1993; 43:876.
- Amato AA, Barohn RJ, Jackson CE, et al. Inclusion body myositis: treatment with intravenous immunoglobulin. Neurology 1994; 44:1516.
- Lindberg C, Trysberg E, Tarkowski A, Oldfors A. Anti-T-lymphocyte globulin treatment in inclusion body myositis: a randomized pilot study. Neurology 2003; 61:260.
- Barohn RJ, Herbelin L, Kissel JT, et al. Pilot trial of etanercept in the treatment of inclusion-body myositis. Neurology 2006; 66:S123.
- Dalakas MC, Rakocevic G, Schmidt J, et al. Effect of Alemtuzumab (CAMPATH 1-H) in patients with inclusion-body myositis. Brain 2009; 132:1536.
- Muscle Study Group. Randomized pilot trial of high-dose betaINF-1a in patients with inclusion body myositis. Neurology 2004; 63:718.
- Muscle Study Group. Randomized pilot trial of betaINF1a (Avonex) in patients with inclusion body myositis. Neurology 2001; 57:1566.
- Kosmidis ML, Alexopoulos H, Tzioufas AG, Dalakas MC. The effect of anakinra, an IL1 receptor antagonist, in patients with sporadic inclusion body myositis (sIBM): a small pilot study. J Neurol Sci 2013; 334:123.
- Patwa HS, Chaudhry V, Katzberg H, et al. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2012; 78:1009.
- Cherin P, Pelletier S, Teixeira A, et al. Intravenous immunoglobulin for dysphagia of inclusion body myositis. Neurology 2002; 58:326.
- Sancricca C, Mora M, Ricci E, et al. Pilot trial of simvastatin in the treatment of sporadic inclusion-body myositis. Neurol Sci 2011; 32:841.
- Amato AA, Sivakumar K, Goyal N, et al. Treatment of sporadic inclusion body myositis with bimagrumab. Neurology 2014; 83:2239.
- Lahouti AH, Amato AA, Christopher-Stine L. Inclusion body myositis: update. Curr Opin Rheumatol 2014; 26:690.
- Rodino-Klapac LR, Haidet AM, Kota J, et al. Inhibition of myostatin with emphasis on follistatin as a therapy for muscle disease. Muscle Nerve 2009; 39:283.
- Kota J, Handy CR, Haidet AM, et al. Follistatin gene delivery enhances muscle growth and strength in nonhuman primates. Sci Transl Med 2009; 1:6ra15.
- Cudkowicz ME, Shefner JM, Simpson E, et al. Arimoclomol at dosages up to 300 mg/day is well tolerated and safe in amyotrophic lateral sclerosis. Muscle Nerve 2008; 38:837.
- Ahmed M, Machado PM, Miller A, et al. Targeting protein homeostasis in sporadic inclusion body myositis. Sci Transl Med 2016; 8:331ra41.
- Peng A, Koffman BM, Malley JD, Dalakas MC. Disease progression in sporadic inclusion body myositis: observations in 78 patients. Neurology 2000; 55:296.
- Cortese A, Machado P, Morrow J, et al. Longitudinal observational study of sporadic inclusion body myositis: implications for clinical trials. Neuromuscul Disord 2013; 23:404.
- 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.
- Price MA, Barghout V, Benveniste O, et al. Mortality and Causes of Death in Patients with Sporadic Inclusion Body Myositis: Survey Study Based on the Clinical Experience of Specialists in Australia, Europe and the USA. J Neuromuscul Dis 2016; 3:67.
- Voermans NC, Vaneker M, Hengstman GJ, et al. Primary respiratory failure in inclusion body myositis. Neurology 2004; 63:2191.
- GOALS OF THERAPY
- OUR APPROACH
- Nonpharmacologic therapy
- - Exercise
- - Ambulation and fall prevention
- - Hand strengthening
- - Speech therapy
- - Nutritional support
- IMMUNOSUPPRESSIVE THERAPY IN SELECTED PATIENTS
- Methotrexate or azathioprine
- AGENTS WITHOUT CLEAR BENEFIT
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS