Treatment of recurrent and resistant 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 two classic forms of inflammatory myopathy. Most patients respond to initial therapy, and some achieve sustained disease control either off all therapy or with low-dose maintenance therapy. There are two additional patterns of response:
●Recurrent disease – After achieving disease control with treatment, some patients experience disease recurrences (flares) during or after the period of medication tapering.
●Resistant disease – In other patients, the disease does not respond entirely to conventional initial approaches to remission induction, and other therapies must be considered after excluding alternative diagnoses.
Approaches to the treatment of recurrent and resistant disease in adults with DM and PM will be reviewed here. The initial therapy of and clinical manifestations of these disorders in adults, the management of the cutaneous manifestations of dermatomyositis, and issues related to DM and PM in children are discussed separately. (See "Initial treatment of dermatomyositis and polymyositis in adults" and "Clinical manifestations of dermatomyositis and polymyositis in adults" and "Initial management of cutaneous dermatomyositis" and "Juvenile dermatomyositis and polymyositis: Epidemiology, pathogenesis, and clinical manifestations" and "Management of refractory cutaneous dermatomyositis" and "Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis".)
Complete discontinuation of treatment is unsuccessful in the majority of patients. For patients who experience disease flares after the achievement of disease control, a variety of clinical scenarios may occur. The following discussion, based primarily on the authors' experience, assumes that prednisone is tapered before attempts to discontinue either azathioprine or methotrexate.
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- RECURRENT DISEASE
- RESISTANT DISEASE
- Intravenous immune globulin
- Mycophenolate mofetil
- Calcineurin inhibitors
- - Cyclosporine
- - Tacrolimus
- Corticotropin injection gel
- Combination methotrexate and azathioprine
- Investigational therapies
- - Ruxolitinib
- EXTRAMUSCULAR DISEASE
- Interstitial lung disease
- Refractory rash
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS
- Recurrent disease
- Resistant disease
- Refractory rash