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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management
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Treatment of Inflammatory Myopathies

The treatment of DM, PM, and necrotizing myopathy is quite similar and can be considered together. Inclusion body myopathy does not respond to these treatments. Corticosteroids are considered first-line treatment. While there are different ways to begin therapy, usually high-dose therapy at 0.75-1.5 mg/kg per day orally is started [2, 5, 6, 8, 12].

Dermatomyositis, polymyositis, and necrotizing myopathy usually respond to treatment with immunosuppressants. There are no recognized effective treatments for IBM at the present time. Treatment with high-dose oral corticosteroids is usually the first-line treatment for DM, PM, and necrotizing myopathy. In severe cases, high-dose IV steroids can be administered (methylprednisolone 1000 mg/day for 3 days). Treatment with steroids and other immunosuppressants is based on experience rather than controlled trials. Typical treatment begins with oral prednisone at 1 mg/kg up to 100 mg a day for 4-6 weeks or until muscle strength improves and the CK values have normalized. It is best to judge the results of treatment on improvement in strength rather than the CK values however. Each treating physician may have his/her protocol as to how the prednisone should be tapered, but a reduction in dose by 10 mg every 4 weeks until the patient is taking 20 mg daily is one way to taper the drug. Once the 20 mg dose is reached, dose should be further reduced by 5 mg daily every 4 weeks until a dose of 10 mg per day is reached. Further reduction by 2.5 mg/day every 4 weeks is then initiated though most patients will require a small maintenance dose long term. An alternate day regimen may be helpful in lessening some of the side effects of the prednisone. It is good practice to obtain a bone density scan prior to initiating corticosteroid therapy. Repeat scans should be performed on a yearly basis. Other recommendations include calcium and vitamin D supplements or treatment with a bisphosphonate. Patients should be instructed to eat a low sodium, high protein, low carbohydrate diet. Blood sugar and potassium levels should be monitored.

Other first-line therapies include physical and occupation therapy and speech and swallowing evaluations [2, 5, 6, 8, 12].

There are numerous second-line therapies for these myopathies as well. Methotrexate in doses ranging from 7.5 to 25 mg per week is often the first of these to be considered. Renal function, liver enzymes, and complete blood counts should be monitored. Patients should receive supplemental folate therapy along with the methotrexate. Since methotrexate can cause pulmonary fibrosis, it should be avoided in patients with interstitial lung disease or who demonstrate presence of anti-Jo antibodies [5].

Other second-line drugs include azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, and rituximab. All have been reported to be useful treatments. In refractory patients, intravenous immunoglobulin (IVIg) at 2 mg/kg/day over

3-5 days has been reported to be beneficial. There are various approaches to the use of these drugs that will not be discussed [12].

 
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