Polymyositis and dermatomyositis medical therapy

Polymyositis and dermatomyositis Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Medical Therapy

• Pharmacologic medical therapies for polymyositis and dermatomyositis include corticosteroids, disease modifying antirheumatic drugs (DMARDs),
• Patients with polymyositis and dermatomyositis might require long term treatment. However, if there are no disease flares during the course of the taper, glucocorticoids could be discontinued at 9 to 12 months. DMARDs might be discontinued at 6 months.

Polymyositis and dermatomyositis

• 1 Stage 1 - Initial treatment
  • 1.1 Corticosteroids
    • Preferred regimen (1): Prednisone 1 mg/kg PO qd (maximum 80 mg daily) for 4-6 weeks and then prednisone should be tapered over 26 weeks to reach 5 mg/d. Prednisone should be tapered by 10 mg every week until 40 mg/day then tapered by 5 mg every week until 20 mg/d then tapered by 2.5 mg every week until 10 mg/day then tapered by 1 mg every two weeks until the patient reaches 5 mg/day.
    • Alternative regimen (1): Methylprednisolone 1000 mg IV qd for 3 days

Note (1): The side effects of corticosteroids include weight gain, redistribution of body fat, thinning of the skin, osteoporosis, cataracts, and muscle weakness. 

• 1.2 Disease modifying antirheumatic drugs (DMARDs) 

• Preferred regimen (1): Methotrexate 15 mg PO every week then increase the daily dose by 2.5 mg increments to 25 mg/week (side effects include hepatotoxicity and pulmonary toxicity.) 
• Preferred regimen (1): Azathioprine 50 mg PO qd for 2 weeks and then increase the daily dose by 50 mg each week to 1.5 mg/kg/day (maximum 2.5 mg/kg/day)
• Preferred regimen (1): Methotrexate
• Preferred regimen (1): Hydroxychloroquine 200-400 mg PO qd (for controlling skin disease)
• Alternative regimen (1): Intravenous immunoglobulin (IVIg)
• Alternative regimen (1): Cyclosporine 
• Alternative regimen (1): Tacrolimus
• Alternative regimen (1): Mycophenolate mofetil
• Alternative regimen (1): Rituximab

• 2 Stage 2 - Resistant
  • 2.1