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Dermatomyositis Medication

  • Author: Alisa N Femia, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Jan 20, 2016
 

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications. In addition to the agents listed below, colchicine, alendronate, and warfarin, amongst other therapies, have been shown to be potentially beneficial for the treatment of calcinosis.

Prednisone is the first-line therapy for muscle involvement in dermatomyositis. The dose is altered according to the response of the patient’s condition.

Antimalarials, particularly hydroxychloroquine, may be useful for cutaneous disease; however, the majority of patients with cutaneous involvement require additional immunomodulatory medications for adequate control. Patients with dermatomyositis are at increased risk for drug eruptions with hydroxychloroquine.

Immunosuppressive/cytotoxic drugs are used as steroid-sparing agents for the muscle disease of dermatomyositis. Methotrexate has been demonstrated to be useful for skin disease, even in the absence of significant muscle disease, and is considered by many experts in the field to be first-line therapy for patients in whom antimalarials fail.[96, 97, 64, 35] Mycophenolate mofetil may also be useful for cutaneous disease. Intravenous immunoglobulin (IVIG) is also beneficial for both cutaneous and muscular involvement.

Biologic therapies have been investigated as potential therapeutic options for dermatomyositis. Existing data regarding anti–tumor necrosis factor alpha therapies have been mixed, and raise potential concerns for use in the dermatomyositis population. Therefore, their widespread use is discouraged until adequate, preferably controlled, studies demonstrate their efficacy and safety.

A small, double-blind, placebo-controlled study of etanercept demonstrated a steroid-sparing effect in five of 11 etanercept-treated patients versus treatment failure in all five placebo-treated patients. The effects were mostly on muscle disease, but some positive effects on skin disease were noted, utilizing the Cutaneous Dermatomyositis Area and Severity Index (CDASI) as a measure. However, two patients in the treated group developed rash, and two developed antinuclear antibodies. One patient in the placebo group developed ovarian cancer, but none in the etanercept group developed cancer during the year-long treatment period.[98]

In addition, one retrospective review of eight patients with dermatomyositis or polymyositis treated with etanercept reported improvement in muscle disease in six patients.[99] However, a case series of five patients with dermatomyositis treated with etancercept found exacerbation of myositis and no improvement in skin disease in any patient.[100]

Infliximab has also been investigated in patients with dermatomyositis; however, results have not been promising. In an open study of 13 patients with refractory myositis, no improvement with infliximab was noted.[101] In addition, one study of infliximab combined with methotrexate for patients with myositis was terminated early due to a high drop-out rate and low inclusion rate.[102]

For these reasons, and because there are several reports of dermatomyositis induced by anti-tumor necrosis factor,[103] anti–tumor necrosis factor therapies should be used only rarely and with caution in patients with severe refractory dermatomyositis.

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Disease-Modifying Antirheumatic Drugs

Class Summary

Disease-modifying antirheumatic drugs (DMARDs) can retard or prevent disease progression and, thus, joint destruction and subsequent loss of function.

Leflunomide (Arava)

 

Immunomodulatory agent; inhibits pyrimidine synthesis, which in turn results in antiproliferative and aniti-inflammatory effects.

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Corticosteroids

Class Summary

The mainstay of therapy for patients with dermatomyositis and muscle involvement is systemic corticosteroids. Cutaneous disease has a variable response to systemic corticosteroids. Disease with pulmonary or cardiac involvement may respond, whereas disease involving esophageal dysfunction usually does not respond. Glucocorticoids may be used topically for cutaneous disease.

Prednisone (Rayos)

 

Prednisone is first-line therapy for dermatomyositis. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN) leukocyte activity. It may be beneficial to use intravenous (IV) pulses; this may be associated with a lower frequency of calcinosis.

Prednisolone (Prelone, Flo-Pred, Millipred)

 

Corticosteroids may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN) leukocyte activity. Shown to improve patients with inflammatory myositis.

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Immunosuppressive Agents

Class Summary

Immunosuppressive agents are used early in the treatment course as steroid-sparing agents. They decrease the risk of steroid-related complications.

Methotrexate (Trexall, Rheumatrex)

 

Methotrexate is used for managing constitutional symptoms. It blocks purine synthesis and 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR), thus increasing anti-inflammatory adenosine concentration at sites of inflammation. Methotrexate ameliorates symptoms of inflammation.

Azathioprine (Imuran, Azasan)

 

Azathioprine is a purine analogue that inhibits purine synthesis, resulting in inhibition of DNA, RNA, and protein synthesis. It may decrease proliferation of immune cells, thereby leading to lower autoimmune activity. It has few, if any, effects on the skin.

Mycophenolate (CellCept, Myfortic)

 

Mycophenolate is useful for both skin and muscle disease. It inhibits purine synthesis and proliferation of human lymphocytes.

Sirolimus (Rapamune)

 

Sirolimus inhibits lymphocyte proliferation by interfering with signal transduction pathways. It binds to immunophilin FK506 binding protein (FKBP) to block the action of mammalian target of rapamycin (mTOR). It is approved by the US Food and Drug Administration (FDA) for prophylaxis against organ rejection in patients receiving allogeneic renal allografts.

A cyclosporine-sparing regimen has recently been FDA-approved for patients with low-to-moderate rejection risk at 2-4 months after transplantation. This regimen allows cyclosporine to be withdrawn, thus significantly decreasing renal toxicity while maintaining a similar antirejection effect.

Rituximab (Rituxan)

 

Rituximab is a third-line choice for the treatment of dermatomyositis. It is a genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. It is an IgG1-kappa immunoglobulin that contains murine light- and heavy-chain variable region sequences and human constant region sequences.

Cyclophosphamide

 

Cyclophosphamide is used for immunosuppression in cases of autoimmune disorders. This agent is chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

Cyclosporine (Gengraf, Neoral, Sandimmune)

 

Cyclosporine (Gengraf, Neoral, Sandimmune)

Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions.

Chlorambucil (Leukeran)

 

Chlorambucil alkylates and cross-links strands of DNA, inhibiting DNA replication and RNA transcription.

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Immune Globulins

Class Summary

High-dose IVIG has been reported to be useful in patients with recalcitrant dermatomyositis.

Immune globulin, intravenous (Gammagard, Carimune NF, Gammaplex, Octagam)

 

IVIG is useful for patients in whom corticosteroids and immunosuppressants have failed.

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Calcium Channel Blockers

Class Summary

Calcium channel blockers may be useful for treating calcinosis.

Diltiazem (Cardizem, Cartia XT, Tiazac, Taztia XT)

 

During depolarization, diltiazem inhibits calcium ions from entering the slow channels and voltage-sensitive areas of vascular smooth muscle and myocardium. Treatment of calcinosis is an off-label use (the mechanism of action is unknown). It appears that other calcium channel blockers are not effective in this setting.

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Calcium Metabolism Modifiers

Class Summary

Bisphosphonates are used to treat hypercalcemia and to decrease calcium loss from bone.

Pamidronate

 

Pamidronate inhibits bone resorption via actions on osteoclasts or on osteoclast precursors, without significant effects on renal tubular calcium handling. It is indicated for treatment of hypercalcemia. Intravenous (IV) pamidronate has been demonstrated in several cases to result in resolution of the calcinosis.

Alendronate (Fosamax, Binosto)

 

Alendronate has been successful in treating the pain of PFD; it may have some benefit in increasing bone mineral density as well. It offers the additional benefit of oral administration. Inhibits bone resorption via actions on osteoclasts or on osteoclast precursors. Has been shown to be potentially beneficial for the treatment of calcinosis

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Antimalarials

Class Summary

Antimalarial agents may be used as steroid-sparing agents to treat skin disease. Hydroxychloroquine is preferred; chloroquine and quinacrine (100 mg/day) are second-line agents. Quinacrine may suppress bone marrow and is distributed by the Centers for Disease Control and Prevention (CDC); blood cell counts should be obtained regularly.

Hydroxychloroquine (Plaquenil)

 

Hydroxychloroquine may allow partial or complete control of the disease. Anecdotal evidence has suggested that morbilliform drug reactions are more common in patients with dermatomyositis than in those with other collagen vascular diseases. Hydroxychloroquine inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.

Chloroquine phosphate (Aralen)

 

Chloroquine phosphate inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.

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Contributor Information and Disclosures
Author

Alisa N Femia, MD Assistant Professor, Ronald O Perelman Department of Dermatology, New York University Medical Center

Alisa N Femia, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Medical Dermatology Society, Rheumatologic Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Ruth Ann Vleugels, MD, MPH Assistant Professor of Dermatology, Harvard Medical School; Associate Physician, Department of Dermatology, Brigham and Women's Hospital; Associate Physician, Department of Immunology and Allergy, Children's Hospital Boston

Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology Foundation

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Acknowledgements

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

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Heliotrope flower, for which characteristic manifestation of dermatomyositis is named.
Heliotrope rash in a woman with dermatomyositis.
Gottron papules and nailfold telangiectasia are present in this patient with dermatomyositis.
These lesions on dorsal hands demonstrate photodistribution of dermatomyositis. Note sparing of interdigital web spaces.
Diffuse alopecia with scaly scalp dermatosis is common in patients with dermatomyositis.
Dermatomyositis is often associated with a poikiloderma in a photodistribution.
Histopathology of dermatomyositis is interface dermatitis.
Calcinosis caused by dermatomyositis in childhood can be observed in patient who had active dermatomyositis 15 years before time of this photograph.
Histopathology of dermatomyositis showing inflammatory myopathic changes with a predominantly perivascular chronic inflammatory infiltrate.
Calcifying panniculitis in patient with dermatomyositis.
Ulceration over dorsal and lateral fingers in patient with dermatomyositis.
Hematoxylin and eosin paraffin section shows polymyositis. Longitudinal section shows dense, chronic, endomysial inflammatory infiltrate. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin frozen section shows polymyositis. Endomysial chronic inflammation is present among intact myofibers that are remarkable only for increased variability of fiber size. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin section shows polymyositis. Patient had dense endomysial inflammation that contains abundance of plasma cells, which can be observed in patients with chronic polymyositis. Two necrotic myofibers, characterized by dense eosinophilic staining, are observed. Focal fatty infiltration of muscle is present in lower left quadrant of photomicrograph. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin section shows polymyositis. Photomicrograph illustrates attack on nonnecrotic myofiber by autoaggressive T lymphocytes. On left, central myofiber is intact. On right, it is obliterated by segmental inflammatory attack. If immunohistochemistry were performed, expected findings would include admixture of CD8 T lymphocytes and macrophages in inflammatory process. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin shows dermatomyositis. In dermatomyositis, inflammation is characteristically perivascular and perimysial. Vessel oriented approximately vertically in center has mild perivascular chronic inflammatory infiltrate. Endothelium is plump; wall is not necrotic. A few lymphocytes in wall of vessel are probably in transit from lumen to external aspect of vessel. Some observers may interpret this finding as vasculitis, but it is certainly neither necrotizing vasculitis nor arteritis. Image courtesy of Roberta J. Seidman, MD.
Hematoxylin and eosin frozen section shows perifascicular atrophy in dermatomyositis. Fascicles in this sample show atrophy, predominantly at periphery, along connective-tissue border. Ischemia is considered to cause perifascicular atrophy. This finding is characteristic of dermatomyositis, mostly associated with juvenile form but also observed in adult form. Image courtesy of Roberta J Seidman, MD.
Immunofluorescence for membrane attack complex of complement (MAC) in dermatomyositis. Bright ring of yellow-green fluorescence at center represents MAC in wall of microvessel. Finding was not present after treatment with steroids. Image courtesy of Roberta J Seidman, MD.
A 47-year-old woman presented with a pruritic, diffuse rash across her upper hands and face that is worsened with sun exposure. ANA testing by outside providers was negative. Her rash was not responsive to topical steroids, and improved with oral prednisone but recurred with tapers beyond 15 mg daily. Diagnosis was dermatomyositis sine myositis. Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD, Division of Rheumatology, University of Colorado Denver School of Medicine.
 
 
 
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