Challenging Cases in Rheumatology and Diseases of the Immune System by Massoud Mahmoudi

Author:Massoud Mahmoudi
Language: eng
Format: epub
Publisher: Springer New York, New York, NY

Treatment. The treatment begins with high-dose corticosteroids intravenously for 3–5 days such as 500–1,000 mg of methylprednisolone if there is severe dysphagia and/or weakness. For outpatient treatment, begin oral prednisone at 60–80 mg daily (1 mg/kg/day) as a single dose for 2–4 weeks followed by 40 mg daily for 2–4 weeks and then tapering by 10 mg every 2–4 weeks until down to 20 mg daily. Once the patient is at 20 mg daily, then the steroid taper should by slowed by 2.5 mg every 2 weeks until completed. At the same time that the patient begins steroids, an immunosuppressive agent should be started. Standard agents used are methotrexate, azathioprine, and mycophenolate mofetil (off-label). Steroid and immunosuppressant doses should be titrated according to patient response and/or side effects encountered.

Summary and conclusion. PM is an inflammatory muscle disease that presents with symmetric, proximal muscle weakness without a rash, and elevated muscle enzymes, an abnormal EMG, and distinct histopathologic features involving an endomysial inflammatory infiltrate composed of cytotoxic CD8+ cells invading non-necrotic fibers on muscle biopsy. Myositis-specific autoantibodies are important for defining clinical phenotypes and determining prognosis. Treatment involves high-dose corticosteroids with a taper and an immunosuppressant agent with doses adjusted according to patient response.

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