eMedicine Specialties > Rheumatology > Systemic Rheumatic Disease
Dermatomyositis: Treatment & Medication
Updated: Apr 14, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Therapy for dermatomyositis (DM) involves general measures and measures to control both the muscle disease and the skin disease. In addition, some patients with dermatomyositis need treatment for other systemic manifestations or complications.
- General treatment
- Bedrest is often valuable in patients with severe muscle inflammation.
- In patients with muscle weakness, especially children, a program of physical therapy is useful to help prevent contractures that can complicate the disease when patients do not fully move their joints.
- For patients with dysphagia, recommending elevating the head of their bed and not eating before bedtime is useful, possibly preventing aspiration pneumonitis. Occasionally, nasogastric tube feeding is needed to increase caloric input.
- Treatment of muscle disease
- The mainstay of therapy for muscle disease of dermatomyositis is systemically administered corticosteroids. Traditionally, prednisone (1-2 mg/kg/d) is administered as initial therapy. Taper slowly to avoid relapse.
- Because most patients develop steroid-related toxic effects, many authorities begin an immunosuppressive or cytotoxic agent early in the course.
- Most clinical and published experience is with the use of methotrexate as a steroid-sparing agent, but azathioprine and mycophenolate mofetil have been used. Results with cyclophosphamide in severe cases have been disappointing.
- When these measures fail, the use of monthly high-dose intravenous immunoglobulin (IVIG) for 6 months has proved beneficial in the short term.
- A recent report has suggested that rituximab, a chimeric antibody directed against CD20+ B cells, may be effective.3 However, other reports have failed to produce positive results for the skin.
- Treatments for skin disease
- Therapy of cutaneous disease of dermatomyositis is often difficult.
- Some patients with dermatomyositis present primarily with skin disease (ie, amyopathic dermatomyositis), while other patients present with a muscle component that is controlled but with significant ongoing skin disease.
- First-line therapy is to recognize that the patient is photosensitive and to prescribe sun avoidance and sun protective measures, including broad-spectrum sunscreens.
- Hydroxychloroquine and chloroquine have been beneficial in small, open-label case studies;7,8 however, roughly 20% of patients with dermatomyositis who are treated with an antimalarial agent develop a drug eruption.
- Methotrexate is useful. Recently, mycophenolate mofetil has been reported to be useful.9,10,11,12,13 Sirolimus may also be of use in some patients.14 In addition, small case series or individual reports of successful management with leflunomide have recently appeared in the literature.15
- IVIG not only benefits the muscle but also clears the skin lesions.
- Calcinosis
- This complication of dermatomyositis affects children and adolescents.
- Some believe that aggressive early treatment of the myositis may aid in preventing calcinosis.
- Once established, the process of calcinosis is debilitating in many patients. Although spontaneous remission is possible, it often occurs after many years.
- The use of the calcium channel blocker diltiazem (240 mg bid) is reportedly associated with gradual resolution of calcinosis in a small number of cases.16 Colchicine, alendronate, and warfarin have also been shown to be potentially beneficial for the resolution of calcinosis. However, the data are not conclusive.
Surgical Care
- Surgical care is usually unnecessary in the management of dermatomyositis.
- Some patients may request surgical removal of local areas of calcinosis.
Consultations
- Rheumatologist
- Dermatologist
- Neurologist
- Medical or surgical oncologist (for patients with cancer)
- Internal medicine specialist or pediatrician (depending on patient age)
- Pulmonologist
Diet
- A well-balanced diet is useful.
- Patients with severe muscle inflammation may need extra protein to balance their loss.
- Patients with dysphagia should avoid eating before bedtime. They may require a special diet depending on the severity of the esophageal dysfunction.
Activity
- Activity in patients with dermatomyositis should be maintained as much as possible; however, avoid vigorous physical training when the myositis is active. Use exercises to maintain the patient's range of motion.
- Recommend sun avoidance and sun protective measures in patients with skin lesions.
Medication
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.
Glucocorticoids
These agents may be used topically for cutaneous disease. The mainstay of therapy for patients with dermatomyositis (DM) 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.
Prednisone (Deltasone, Orasone, Meticorten)
First-line therapy for dermatomyositis. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. May be beneficial to use IV pulses and may be associated with lower frequency of calcinosis.
Adult
1-2 mg/kg PO qd
Pediatric
Administer as in adults
Coadministration with estrogens may decrease prednisone clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase the metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
No absolute contraindication; severe bacterial, viral, or fungal infection; active peptic ulcer disease; diabetes mellitus
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Perform general medical assessment of patient before treatment (DEXA scan may be useful); check blood pressure; ophthalmologic evaluation may assess and prevent ocular complications; skin tests and/or chest radiograph detects potential for reactivation of tuberculosis; abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression in children, and infections may occur with glucocorticoid use
Immunosuppressive agents
These agents are used early in the course as steroid-sparing agents. They decrease the risk of steroid-related complications.
Methotrexate (Folex, Rheumatrex)
Has benefits in both muscle and skin disease. Unknown mechanism of action in treatment of inflammatory reactions. May affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Antimetabolite that inhibits DNA synthesis and cell reproduction in malignant cells. May suppress immune system. Satisfactory response 3-6 wk following administration. Adjust dose gradually to attain satisfactory response.
Adult
10-30 mg/wk PO/SC
Pediatric
10-25 mg/wk PO/SC
PO aminoglycosides may decrease absorption and blood levels of concurrent PO MTX; charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; indomethacin can increase MTX plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, may increase effects and toxicity of MTX; may increase plasma levels of thiopurines
Documented hypersensitivity; alcoholism; hepatic insufficiency; immunodeficiency syndromes; blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia)
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Avoid pregnancy during therapy and probably for at least 3 mo after cessation; stop excessive alcohol intake; monitor CBC counts and liver enzymes during therapy; discontinue if significant drop in blood counts occurs; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with MTX; caution in diabetes and obesity; concomitant administration of folic acid 1 mg/d can help prevent adverse GI effects without diminishing effectiveness
Azathioprine (Imuran)
Purine analog inhibiting purine synthesis results in inhibition of DNA, RNA, and protein synthesis. May decrease proliferation of immune cells, which results in lower autoimmune activity. Few, if any, effects on skin.
Adult
2-3 mg/kg/d PO/IV
Pediatric
Administer as in adults
Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine
Documented hypersensitivity; deficiency of thiopurine methyltransferase; active infection; severe cytopenias (relative contraindication)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Measuring thiopurine methyltransferase prior to therapy may avoid hematologic toxicity; fever may be idiosyncratic reaction; may be increased risk of lymphoproliferative disorders with long-term therapy; increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur
Mycophenolate (CellCept)
Useful for both skin and muscle disease. Inhibits purine synthesis and proliferation of human lymphocytes.
Adult
1-1.5 g PO bid
Pediatric
Not established; 15-23 mg/kg PO bid suggested
May elevate levels of acyclovir and ganciclovir; antacids and cholestyramine decrease absorption, reducing levels (do not administer together); probenecid may increase levels of mycophenolate; salicylates may increase toxicity of mycophenolate; other immunosuppressives may increase risk of infection or malignancy
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
May increase risk of lymphoma in patients on long-term therapy; GI intolerance may occur with high doses; increases risk for infection; increases toxicity in renal impairment; caution in active peptic ulcer disease
Sirolimus (Rapamune)
Inhibits lymphocyte proliferation by interfering with signal transduction pathways. Binds to immunophilin FKBP to block action of mTOR. FDA approved for prophylaxis of 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 mo following transplantation. This regimen allows for cyclosporine to be withdrawn, thus significantly decreasing renal toxicity while maintaining similar antirejection effect.
Adult
Loading dose of 6 mg PO, followed by 2 mg/d PO as single dose; trough blood concentrations >8 ng/mL correlated with immunosuppressive activity
Cyclosporine-sparing regimen in de novo transplant recipients: Coadministered with cyclosporine and corticosteroids following transplantation; in patients with low-to-moderate immunologic risk, withdraw cyclosporine 2-4 mo following transplant, then increase sirolimus dose and monitor blood levels to maintain optimal dose
Pediatric
Not established
Levels/toxicity may increase with diltiazem, nicardipine, clotrimazole, verapamil, erythromycin, ketoconazole, itraconazole, fluconazole, bromocriptine, grapefruit juice, metoclopramide, methylprednisolone, danazol, cyclosporine, cimetidine, and clarithromycin; levels may decrease with rifabutin, rifampin, phenobarbital, phenytoin, and carbamazepine
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
May exacerbate hyperlipidemia and thrombocytopenia; caution with hepatic impairment (decrease maintenance dose by one third); monitor blood sirolimus blood levels in pediatric patients, in patients with hepatic impairment, during coadministration of strong CYP450 3A4 inducers or inhibitors, or if cyclosporine dosing is markedly reduced or discontinued
Not recommended for use in de novo liver or lung transplantation; coadministration with cyclosporine or tacrolimus in patients who have undergone liver transplantation increases hepatic artery thrombosis risk; bronchial anastomotic dehiscence, most fatal, has been reported in de novo lung transplantation when sirolimus has been part of the immunosuppressive regimen
Rituximab (Rituxan)
This is a third-line choice for the treatment of dermatomyositis. It is an antibody genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. Antibody is an IgG1-kappa immunoglobulin that contains murine light- and heavy-chain variable region sequences and human constant region sequences.
Adult
375 mg/m2 IV qwk for 4 doses (days 1, 8, 15, and 22)
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypotension, bronchospasm, and angioedema may occur; discontinue treatment if life-threatening cardiac arrhythmias occur
Immune globulins
High-dose IV immunoglobulin has been reported to be useful in patients with recalcitrant dermatomyositis.
Immune globulin intravenous (Gamimune, Gammagard, Sandoglobulin)
For patients in whom corticosteroids and immunosuppressives have failed.
Adult
1 g/kg for 2 days then monthly for at least 6 mo
Pediatric
Administer as in adults
None reported
Documented hypersensitivity; IgA deficiency; anti-IgE/IgG antibodies
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Consider checking serum IgA before administering IVIG and using IgA-depleted IVIG (G-Gard-SD), if indicated; IVIG may increase serum viscosity and thromboembolic events; infusions may increase risk of migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or petechiae (2-30 d postinfusion); increased risk of renal tubular necrosis in elderly patients and in patients with diabetes, volume depletion, and preexisting kidney disease; laboratory result changes associated with infusions include elevated antiviral or antibacterial antibody titers for 1 mo, 6-fold increase in ESR for 2-3 wk, and apparent hyponatremia
Calcium channel blockers
These agents may be useful to treat calcinosis.
Diltiazem (Cardizem, Tiamate, Dilacor, Tiazac)
During depolarization, inhibits calcium ions from entering the slow channels and voltage-sensitive areas of vascular smooth muscle and myocardium. Off-label use (MOA unknown). Appears that other calcium channel blockers are not effective.
Adult
240-480 mg/d PO divided bid
Pediatric
Administer as in adults
May increase carbamazepine, digoxin, cyclosporine, and theophylline levels; when administered with amiodarone, may cause bradycardia and a decrease in cardiac output; when administered with beta-blockers, may increase cardiac depression; cimetidine may increase diltiazem levels
Documented hypersensitivity; severe CHF; sick sinus syndrome; second- or third-degree AV block; hypotension (<90 mm Hg systolic)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in impaired renal or hepatic function; may increase LFT levels; hepatic injury may occur
Antimalarial agents
These agents may be used as steroid-sparing agents to treat skin disease. Hydroxychloroquine is preferred; chloroquine and quinacrine (100 mg/d) 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)
May allow partial or complete control of the disease. Anecdotes have suggested that morbilliform drug reactions are more common in patients with dermatomyositis than in those with other collagen vascular diseases. Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.
Adult
200-400 mg/d PO; not to exceed 6.5 mg/kg/d
Pediatric
6-7 mg/kg/d PO
Serum levels increase with cimetidine; magnesium trisalicylate may decrease absorption
Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform regular ophthalmologic examinations to evaluate for possible retinopathy (rare); caution in hepatic disease, G-6-PD deficiency, psoriasis, porphyria, and myopathy
Chloroquine phosphate (Aralen phosphate)
Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.
Adult
250-500 mg PO qd
Pediatric
Not established; 4 mg/kg/d PO can be used
May increase serum levels of chloroquine (possibly other 4-aminoquinolones); magnesium trisalicylate may decrease absorption of 4-aminoquinolones
Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long-term use in children; perform periodic ophthalmologic examinations to evaluate for possible retinopathy; myopathy
More on Dermatomyositis |
| Overview: Dermatomyositis |
| Differential Diagnoses & Workup: Dermatomyositis |
 Treatment & Medication: Dermatomyositis |
| Follow-up: Dermatomyositis |
| Multimedia: Dermatomyositis |
| References |
| Further Reading |
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Keywords
dermatomyositis, idiopathic inflammatory myopathy, IIM, dermatomyositis sine myositis, amyopathic dermatomyositis, ADM, juvenile dermatomyositis, childhood dermatomyositis, DM, polymyositis, PM, DM-sine myositis, myositis with malignancy, childhood DM, childhood PM, myositis, postmyopathic DM, postmyopathic dermatomyositis, complement-mediated vascular inflammation, terminal attack complex, calcinosis, heliotrope rash, Gottron papules
