Dermatomyositis Medication

  • Author: Jeffrey P Callen, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Oct 12, 2011
 

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 have been shown to be potentially beneficial for the treatment of calcinosis.

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

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. Mycophenolate mofetil may also be useful for cutaneous disease.

Antimalarials, particularly hydroxychloroquine, may be useful for cutaneous disease. Patients with dermatomyositis may be at increased risk for drug eruptions.

Intravenous immunoglobulin (IVIG) is used in patients in whom therapy with corticosteroids and immunosuppressants fails. This agent is useful for both the skin and the muscles.

Biologic therapies, including rituximab and tumor necrosis factor (TNF) antagonists might be useful. However, their widespread use is discouraged until adequate, preferably controlled, studies demonstrate their efficacy and safety. A recent, small, double-blind, placebo-controlled study of etanercept has demonstrated a steroid-sparing effect among 5 of 11 etanercept-treated patients versus treatment failure in all 5 placebo-treated patients. The effects were mostly on muscle disease, but some positive effects on skin disease were noted, utilizing the CDASI as a measure. However, 2 patients developed rash in the treated group, and 2 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. While this is a small study, it does suggest that etanercept might have a role in the treatment of dermatomyositis.[51]

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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.

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Prednisone

 

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.

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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.

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Methotrexate (Trexall, Rheumatrex)

 

Methotrexate has benefits in both muscle and skin disease. Its mechanism of action in treatment of inflammatory reactions is unknown. It may affect immune function. Methotrexate ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). It is an antimetabolite that inhibits DNA synthesis and cell reproduction in malignant cells. A satisfactory response is typically seen 3-6 weeks after administration. Adjust the dose gradually to attain satisfactory response.

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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.

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Mycophenolate (CellCept, Myfortic)

 

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

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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.

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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.

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Immune Globulins

Class Summary

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

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Immune globulin, intravenous (Gammagard, Carimune, 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.

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Diltiazem (Cardizem, Dilacor XR, 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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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.

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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.

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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

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, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; 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, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Additional Contributors

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

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.

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

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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.
 
 
 
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