eMedicine Specialties > Neurology > Neuromuscular Diseases

Inclusion Body Myositis: Treatment & Medication

Author: Helen C Lin, MD, Assistant Professor of Neurology, Medical College of Wisconsin, Milwaukee
Coauthor(s): Paul E Barkhaus, MD, Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Administration Medical Center; Michael P Collins, MD, Associate Professor, Department of Neurology, Medical College of Wisconsin; M Isabel Periquet Collins, MD, Assistant Professor, Department of Neurology, Medical College of Wisconsin
Contributor Information and Disclosures

Updated: Sep 3, 2009

Treatment

Medical Care

No definitive treatment has been proven effective for s-IBM.

Early anecdotal reports documented the failure of patients to respond to steroids, methotrexate, azathioprine, and cyclophosphamide. Subsequent clinical studies of various immunosuppressive or immunomodulatory therapies have largely been disappointing. Individual responses, functional improvement, or mild regional improvement in strength have been reported, but sustained remission and improvement in whole-body strength have not been demonstrated.

  • An open-label study of high-dose prednisone in 8 patients showed no improvement in strength or functional disability scores despite a decrease in creatine phosphokinase (CPK) and inflammatory cell infiltration. Posttreatment muscle biopsy samples showed increased vacuole formation and amyloid deposition, suggesting that mechanisms other than the inflammatory response play a role in disease propagation.85
  • A randomized, controlled study of oxandrolone in 19 patients reported a regional improvement in upper extremity strength, but only borderline improvement in whole-body strength.86
  • A randomized, controlled study of methotrexate in 44 patients likewise showed no improvement in strength despite a significant decrease in CPK levels.87
  • An early small, uncontrolled study reported improvement in strength in 4 patients following intravenous immunoglobulin (IVIg) treatment.88 However, subsequent larger and placebo-controlled studies have failed to duplicate these results.89,21,90 Two studies suggest some benefit in patients with severe dysphagia.91,92 A subsequent controlled study of IVIg in combination with prednisone likewise showed no treatment response despite a reduction in endomysial inflammation.93
  • An open-label, randomized study of anti-T-lymphocyte globulin treatment followed by 12 months of oral methotrexate (vs methotrexate alone) reported regional improvement in distal upper extremity strength, but continued deterioration of the proximal muscle groups.94
  • Beta interferon-1a at standard (30 µg IM/wk) and high-dosage (60 µg IM/wk) regimens were found to be well tolerated but produced no significant improvement in muscle strength or muscle mass.95,96
  • A pilot trial of etanercept, a tumor necrosis factor (TNF) alpha blocker, did not show significant benefit in composite muscle strength scores at 6 months. However, with 12 months of treatment, slight improvement in grip strength was noted.97
  • A study of alemtuzumab, a T-cell–depleting monoclonal antibody, involved 13 patients who underwent infusion of 0.3 mg/kg/d for 4 days. It reported slowed disease progression, improvement of strength in some patients, and reduction in endomysial inflammation.98 This preliminary study holds promise for future studies.
  • Follistatin, an antagonist of the myostatin pathway, has been shown to produce a dramatic increase in muscle mass in animals.99 These results are promising for future gene therapy trials to improve muscle mass in patients with neuromuscular disease.
  • Arimoclomol, a heat shock protein (HSP) coinducer may slow down the process of protein misfolding and aggregation. A study of its safety and efficacy in IBM is underway.
  • Lithium is an inhibitor of the glycogen synthase kinase (GSK) enzyme, the latter of which is involved in the development of phosphorylated tau (p-tau). A study is being conducted to examine its efficacy in slowing muscle degeneration in IBM.
  • Empiric therapies include coenzyme Q10, carnitine, and antioxidants. They may provide benefit to some patients, but, to date, none of these has been studied in a controlled clinical trial.
  • Routine follow-up visits at intervals contingent upon the progression and severity of involvement are indicated to assess the patient's strength, tolerance of exertion, and compromise in occupation or activities of daily living. Hicks has outlined a strategy for care of patients with inflammatory myopathies, including s-IBM.100

Surgical Care

  • Muscle biopsy is performed for diagnosis.
  • Severe dysphagia may require cricopharyngeal myotomy or placement of a gastrostomy tube. Chemodenervation with botulinum toxin A injection into the upper esophageal sphincter has also been shown to be of benefit.101

Consultations

  • Depending on degree of weakness, input from physical therapy or physiatry may be useful in optimizing the patient's abilities.
  • If dysphagia occurs, referral to a speech therapist would be of benefit for instruction regarding swallowing techniques and aspiration precautions. In patients with severe dysphagia, referral to ear, nose, and throat (ENT) specialist is indicated for consideration of botulinum toxin injections or cricopharyngeal myotomy.

Diet

No dietary modification is required in most cases unless symptomatic dysphagia occurs.

Activity

Appropriate activity level depends on the condition of the patient.

Strength training and exercise regimens have been subjects of debate, given concerns that physical activity might instigate increased muscle breakdown and inflammation. However, 3 recent studies have shown that an exercise program can be instituted safely.102,81,103 In the study by Arnardottir, 6 of 7 patients reported a subjective positive effect on muscle function after a 12-week exercise regimen.81 No improvement or deterioration in strength was observed, and no increase in inflammation was noted in pretreatment and posttreatment muscle biopsy specimens. In the study by Johnson, 7 patients underwent a combined aerobic and functional exercise regimen.103 The patients exhibited improved aerobic and functional exercise capacity and strength without significant increase in creatine kinase.

Medication

Different immunosuppressive treatment regimens have been reported (see Treatment/Medical Care). The general consensus is that none of these treatments has proven benefits.

Despite lack of responsiveness to treatment, patients may be offered a trial of steroids in the hope of at least slowing progression, but this is controversial (see Prognosis). Given the variety of empiric treatment protocols, the reader is referred directly to these reports for details.

More on Inclusion Body Myositis

Overview: Inclusion Body Myositis
Differential Diagnoses & Workup: Inclusion Body Myositis
Treatment & Medication: Inclusion Body Myositis
Follow-up: Inclusion Body Myositis
Multimedia: Inclusion Body Myositis
References
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Further Reading

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Keywords

sporadic inclusion body myositis, s-IBM, hereditary inclusion body myopathies, h-IBM, idiopathic inflammatory myopathies, polymyositis, PM, dermatomyositis, DM, myositis, amyloid precursor protein, APP, alien molecules, AB oligomers, alpha beta oligomers, endoplasmic reticulum, ER, unfolded protein response, UPR, heat shock protein 70, phosphorylated tau, p-tau

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Contributor Information and Disclosures

Author

Helen C Lin, MD, Assistant Professor of Neurology, Medical College of Wisconsin, Milwaukee
Helen C Lin, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Paul E Barkhaus, MD, Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Administration Medical Center
Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association
Disclosure: Nothing to disclose.

Michael P Collins, MD, Associate Professor, Department of Neurology, Medical College of Wisconsin
Michael P Collins, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, Peripheral Nerve Society, and World Muscle Society
Disclosure: Nothing to disclose.

M Isabel Periquet Collins, MD, Assistant Professor, Department of Neurology, Medical College of Wisconsin
M Isabel Periquet Collins, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine
Disclosure: Nothing to disclose.

Medical Editor

Dianna Quan, MD, Associate Professor of Neurology, Director, Electromyography Laboratory, University of Colorado Health Sciences Center
Dianna Quan, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: e-medicine Honoraria Other

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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