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 clinical 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:
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- 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