Juvenile Dermatomyositis Treatment & Management

Updated: May 12, 2021
  • Author: Ann M Reed, MD; Chief Editor: Lawrence K Jung, MD  more...
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Treatment

Approach Considerations

A multidisciplinary approach is required to prevent and reduce long-term morbidity in juvenile dermatomyositis (JDM). Various therapies are used to treat skin manifestations of JDM. For active muscle disease, oral corticosteroids are the mainstay of treatment. Second-line agents are routinely added for steroid-sparing effects and for recalcitrant or refractory disease.

Cyclophosphamide at a dose of 0.5-1 g/m2 may be given with bladder protection on a monthly basis and adjusted for leukopenia for patients with significant morbidity such as skin or GI ulcerations, respiratory disease, or both. [94]

For patients with dysphonia, a referral to a speech therapist is appropriate. Children with dysphagia should meet with an occupational therapist for instruction on food consistency and proper positioning of the head, chin, and tongue. [2]

Because of the rarity of the disease, patients with JDM should be transferred to a tertiary care center for experienced clinical care in the initial course of the disease and for periodic follow-up. Patients should be followed on a regular basis, with monitoring of muscle enzymes and muscle strength every 3-6 months. Remission occurs when stable improvement or normalization of muscle strength and muscle enzymes is observed over 6 months.

Consultations

Consultations may be indicated with the following:

  • Pediatric rheumatologist

  • Dermatologist

  • Pediatric neurologist

  • Physical therapist

  • Occupational therapist

  • Speech therapist

  • Orthopedic surgeon

  • Pediatric endocrinologist

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Treatment of Skin Disease

Photosensitive rashes in patients with JDM may be exacerbated by sunlight exposure; sun avoidance, and judicious use of sunscreens that protect against ultraviolet A and B rays. U

Topical corticosteroids are used to treat skin manifestations of JDM. Hydroxychloroquine at a dose no greater than 5-7 mg/kg/day may be beneficial. Topical tacrolimus (0.1%) has been used to treat cutaneous disease, [95] but this use is falling out of favor. Methotrexate, which is an established second-line agent for muscle involvement in JDM, may also be effective for cutaneous manifestations. [96]

Calcinosis cutis

Medical therapy for calcinosis in JDM has been controversial due to inconsistent responses. Diltiazem at doses of 240-480 mg (3-6 mg/kg/day) has been used with varying success rates. [2, 97, 98] Other agents used with little success include bisphosphonates, aluminum hydroxide, probenecid, and local corticosteroid injections. [98] Calcinosis cutis may regress over time.

Areas of calcinosis that cause significant disfigurement, pain, or compromised physical function may be resected surgically. Resection is not usually recommended, however, as areas of resection may be complicated by draining sinuses, infections, and ulcerations; the calcinosis may recur.

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Treatment of Muscle Disease

For active muscle disease, oral corticosteroids are the mainstay of treatment. High doses (1-2 mg/kg/day) are initially used until improvement or for 4-6 weeks, with a slow taper to avoid relapse. Weaning of steroids often occurs over 1-2 years. For refractory or severe disease, pulse therapy with intravenous methylprednisolone is used at a dose of 30 mg/kg, with a maximum dose of 1 g daily for 3 days with efficacy. [99] Calcium and vitamin D supplementation is recommended for patients on long-term corticosteroid therapy.

Second-line agents are routinely added for steroid-sparing effects and for recalcitrant or refractory disease. Methotrexate has been the most widely accepted agent and is used at doses of 10-20 mg/m2 per week orally or subcutaneously with 1 mg/d of folic acid supplementation.

Methotrexate administration may be started at the outset of severe disease (eg, moderate to severe weakness, manifestations of vasculopathy) or started if patients fail to respond to high-dose corticosteroids within 6 weeks. The use of methotrexate in conjunction with a tapering course of prednisone may be effective in reducing the long-term cumulative dose of corticosteroids. [100, 98]

Cyclosporin A has been used as an alternative, effective, steroid-sparing agent in JDM. [101] Preliminary data also suggest that the use of cyclosporine A in combination with methotrexate may be associated with further improvement in clinical outcome.

Intravenous immunoglobulin (IVIG) may also be used in patients with JDM who are steroid-resistant or steroid-dependent at a dose of 1 g/kg on 2 consecutive days, or 2 g/kg in one day, then every month thereafter, generally for a 6-month course. Other second-line agents include azathioprine and mycophenolate mofetil. [98]

Biologic agents such as the anti–tumor necrosis factor agents have had mixed results. More recently, infliximab has shown major clinical benefit in 5 patients with refractory JDM. [102] Another promising agent for JDM is the anti-CD20 monoclonal antibody rituximab. [103] In one trial of adults with refractory polymyositis and adults and children with refractory dermatomyositis, 200 patients were randomized to either rituximab early or rituximab late. Glucocorticoid and immunosuppressive therapy were allowed at entry. While no significant differences in outcomes between treatment groups were observed, 83% of refractory adult and juvenile myositis patients met the International Myositis Assessment and Clinical Studies Group definition of improvement. [104]

Rehabilitation

Rehabilitation plays an active role in the first-line management of myositis to restore and maintain muscle strength and endurance and to prevent contractures. If the myositis is active, then passive range of motion exercises are recommended; pool therapy may also be helpful.

When the disease is stabilized, isometric and isotonic exercises have been recommended. [2] However, one study indicated that an intensive muscular training program involving resistance training may be beneficial and safe without worsening of muscle inflammation. [105]

For patients with severe contractures, physical and occupational therapy with assistive devices is recommended.

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Diet and Activity

No special diet is advised for most patients with JDM, although calcium and vitamin D supplementation are usually recommended for those using corticosteroids. Patients who have dysphagia may have a consistency-limited diet as recommended by a physical therapist.

Vigorous physical activity should be avoided if the child has instabilities, severe fatigue, or concerns of falling or aspiration. Bed rest is not indicated. Protective measures against sun exposure are recommended.

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