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 in adults" and "Juvenile dermatomyositis and polymyositis: Epidemiology, pathogenesis, and clinical manifestations" and "Management of refractory cutaneous dermatomyositis in adults" 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.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Benveniste O, Guiguet M, Freebody J, et al. Long-term observational study of sporadic inclusion body myositis. Brain 2011; 134:3176.
- Oddis CV, Reed AM, Aggarwal R, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum 2013; 65:314.
- Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum 2005; 52:601.
- Brulhart L, Waldburger JM, Gabay C. Rituximab in the treatment of antisynthetase syndrome. Ann Rheum Dis 2006; 65:974.
- Noss EH, Hausner-Sypek DL, Weinblatt ME. Rituximab as therapy for refractory polymyositis and dermatomyositis. J Rheumatol 2006; 33:1021.
- Lambotte O, Kotb R, Maigne G, et al. Efficacy of rituximab in refractory polymyositis. J Rheumatol 2005; 32:1369.
- Mahler EA, Blom M, Voermans NC, et al. Rituximab treatment in patients with refractory inflammatory myopathies. Rheumatology (Oxford) 2011; 50:2206.
- Rios Fernández R, Callejas Rubio JL, Sánchez Cano D, et al. Rituximab in the treatment of dermatomyositis and other inflammatory myopathies. A report of 4 cases and review of the literature. Clin Exp Rheumatol 2009; 27:1009.
- Valiyil R, Casciola-Rosen L, Hong G, et al. Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: a case series. Arthritis Care Res (Hoboken) 2010; 62:1328.
- 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.
- 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.
- Cherin P, Pelletier S, Teixeira A, et al. Results and long-term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: an open study with thirty-five adult patients. Arthritis Rheum 2002; 46:467.
- Danieli MG, Pettinari L, Moretti R, et al. Subcutaneous immunoglobulin in polymyositis and dermatomyositis: a novel application. Autoimmun Rev 2011; 10:144.
- Schneider C, Gold R, Schäfers M, Toyka KV. Mycophenolate mofetil in the therapy of polymyositis associated with a polyautoimmune syndrome. Muscle Nerve 2002; 25:286.
- Rowin J, Amato AA, Deisher N, et al. Mycophenolate mofetil in dermatomyositis: is it safe? Neurology 2006; 66:1245.
- Pisoni CN, Cuadrado MJ, Khamashta MA, et al. Mycophenolate mofetil treatment in resistant myositis. Rheumatology (Oxford) 2007; 46:516.
- Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology (Oxford) 2005; 44:386.
- Grau JM, Herrero C, Casademont J, et al. Cyclosporine A as first choice therapy for dermatomyositis. J Rheumatol 1994; 21:381.
- Vencovský J, Jarosová K, Machácek S, et al. Cyclosporine A versus methotrexate in the treatment of polymyositis and dermatomyositis. Scand J Rheumatol 2000; 29:95.
- Qushmaq KA, Chalmers A, Esdaile JM. Cyclosporin A in the treatment of refractory adult polymyositis/dermatomyositis: population based experience in 6 patients and literature review. J Rheumatol 2000; 27:2855.
- Maeda K, Kimura R, Komuta K, Igarashi T. Cyclosporine treatment for polymyositis/dermatomyositis: is it possible to rescue the deteriorating cases with interstitial pneumonitis? Scand J Rheumatol 1997; 26:24.
- Oddis CV, Sciurba FC, Elmagd KA, Starzl TE. Tacrolimus in refractory polymyositis with interstitial lung disease. Lancet 1999; 353:1762.
- Wilkes MR, Sereika SM, Fertig N, et al. Treatment of antisynthetase-associated interstitial lung disease with tacrolimus. Arthritis Rheum 2005; 52:2439.
- Takada K, Nagasaka K, Miyasaka N. Polymyositis/dermatomyositis and interstitial lung disease: a new therapeutic approach with T-cell-specific immunosuppressants. Autoimmunity 2005; 38:383.
- Ochi S, Nanki T, Takada K, et al. Favorable outcomes with tacrolimus in two patients with refractory interstitial lung disease associated with polymyositis/dermatomyositis. Clin Exp Rheumatol 2005; 23:707.
- Bombardieri S, Hughes GR, Neri R, et al. Cyclophosphamide in severe polymyositis. Lancet 1989; 1:1138.
- Sinoway PA, Callen JP. Chlorambucil. An effective corticosteroid-sparing agent for patients with recalcitrant dermatomyositis. Arthritis Rheum 1993; 36:319.
- Yamasaki Y, Yamada H, Yamasaki M, et al. Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis. Rheumatology (Oxford) 2007; 46:124.
- Levine T. Treating refractory dermatomyositis or polymyositis with adrenocorticotropic hormone gel: a retrospective case series. Drug Des Devel Ther 2012; 6:133.
- Villalba L, Hicks JE, Adams EM, et al. Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum 1998; 41:392.
- Hornung T, Janzen V, Heidgen FJ, et al. Remission of recalcitrant dermatomyositis treated with ruxolitinib. N Engl J Med 2014; 371:2537.
- 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