Initial treatment of dermatomyositis and polymyositis in adults
- 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
Dermatomyositis (DM) and polymyositis (PM) are classified as idiopathic inflammatory myopathies. Defining the optimal treatment regimens for these disorders has been difficult because of the rarity of these disorders, their highly complex clinical phenotypes, and the limited number of randomized, double-blind clinical trials [1-3].
In addition, understanding of the inflammatory myopathies has evolved over the years, but disease classification schemes have not kept pace. Newer classifications, based upon histologic and serologic distinctions between the different disorders, may help define the natural history of these diseases better, may assist in the design of well-conceived randomized clinical trials, and may lead to the development of rational therapies [4-6].
The initial therapy of DM and PM will be reviewed here. The treatment of recurrent or resistant disease, the clinical manifestations and approach to diagnosis of these disorders in adults, the management of cutaneous manifestations of dermatomyositis, and issues related to DM and PM in children are discussed separately. (See "Treatment of recurrent and resistant dermatomyositis and polymyositis in adults" and "Clinical manifestations of dermatomyositis and polymyositis in adults" and "Initial management of cutaneous dermatomyositis" and "Management of refractory cutaneous dermatomyositis" and "Juvenile dermatomyositis and polymyositis: Epidemiology, pathogenesis, and clinical manifestations" and "Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis".)
PREDICTORS OF OUTCOME
The severity of disease in dermatomyositis (DM) and polymyositis (PM) is highly variable, ranging from mild weakness that responds readily to treatment to muscle dysfunction associated with a relentless downhill course that is unresponsive to all treatment modalities. Some clinical and laboratory features are associated with a poorer prognosis.
Clinical predictors — Most studies that have investigated prognosis in the inflammatory myopathies did not distinguish between DM and PM. Many also did not recognize inclusion body myositis, a common mimic of PM that is associated with a worse prognosis but was not defined until the 1980s. (See "Clinical manifestations and diagnosis of inclusion body myositis".)
- Bunch TW, Worthington JW, Combs JJ, et al. Azathioprine with prednisone for polymyositis. A controlled, clinical trial. Ann Intern Med 1980; 92:365.
- Dalakas MC, Illa I, Dambrosia JM, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 1993; 329:1993.
- Miller FW, Leitman SF, Cronin ME, et al. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med 1992; 326:1380.
- Troyanov Y, Targoff IN, Tremblay JL, et al. Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 French Canadian patients. Medicine (Baltimore) 2005; 84:231.
- Hoogendijk JE, Amato AA, Lecky BR, et al. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10-12 October 2003, Naarden, The Netherlands. Neuromuscul Disord 2004; 14:337.
- Isenberg DA, Allen E, Farewell V, et al. International consensus outcome measures for patients with idiopathic inflammatory myopathies. Development and initial validation of myositis activity and damage indices in patients with adult onset disease. Rheumatology (Oxford) 2004; 43:49.
- 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.
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- PREDICTORS OF OUTCOME
- Clinical predictors
- Type of myositis
- Myositis-specific autoantibodies
- INITIAL THERAPY
- Systemic glucocorticoids
- - General approach
- - Initial glucocorticoid therapy
- - Assessing treatment response
- - Glucocorticoid tapering
- - Alternate-day glucocorticoid tapers
- - Glucocorticoid responses
- - Apparent glucocorticoid failures
- GLUCOCORTICOID-SPARING AGENTS
- - Thiopurine methyltransferase testing
- - Dosing and monitoring
- - Adverse effects
- - Dosing
- - Adverse effects
- Other agents
- DURATION OF THERAPY
- LONG-TERM FOLLOW-UP
- RECURRENT AND RESISTANT DISEASE
- GENERAL MEASURES AND EXTRAMUSCULAR MANIFESTATIONS
- Aspiration risk
- Skin disease and avoidance of sunlight
- - Pruritus
- Osteoporosis prevention
- Opportunistic infections
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS
- Predictors of outcome
- Initial therapy
- Glucocorticoid-sparing agents
- General treatment measures