難治性若年性皮膚筋炎にSteroid/tacrolimus併用が著効した一例

抄録

    The patient was an18-year-old female who first showed high CPK levels in August 2000 (when she was 12years old). Muscle biopsy confirmed dermatomyositis. Together with skin rash, she was diagnosed with juvenile dermatomyositis. She was treated with oral prednisolone (PSL) at a daily dose of 60 mg (≒2 mg/kg). Cyclosporine A (140 mg daily) was added for complicating interstitial pneumonia. However, her disease frequently relapsed when she had the common cold or other triggering factors. Various second-line treatments, such as intravenous immunoglobulin (IVIg) (45 g; 2 sessions), monthly IVCY (700 mg/body; 22 sessions), pulse methylprednisolone (1 g; 9 sessions), azathioprine (AZP, 50 mg daily), methotrexate (MTX, 16 mg weekly) and mycophenolate mofetil (3 g daily), were attempted for relapses. However, her flare never placed under good control. On the first visit to our clinic at the age of 18 years (February 2007), she showed a significantly increased level of serum CPK. Despite the combination of oral PSL (20 mg daily), AZP (150 mg daily) and MTX (16 mg weekly), her CPK reached8, 154 IU/L. Thus, AZP and MTX were substituted with tacrolimus (TAC), which had never been used, and pulse therapy with methylprednisolone (m-PSL) followed by oral m-PSL (48 mg daily). Her serum levels of myogenic enzymes were normalized after this treatment and no relapse has been observed for a year.
    Recently, there have been domestic and overseas studies describing the effectiveness of TAC on myositis. This is another case demonstrating the efficacy of the combination therapy of steroid hormone and TAC on dermatomyositis resistant to various immunosuppressants.