Q: My 70-year old sister has had dermatomyositis for more than a year. Her muscles, joints, skin and lungs have been affected. She has not responded well to treatment with steroids. Are there any alternatives? I read somewhere about transfusions of immune globulin. Is this a reputable treatment?
A: Dermatomyositis is a condition of unknown cause marked by rash and inflammation of the muscles. Muscles become weak and sore. Other organs, including the lungs and joints, can also be inflamed.
Initial treatment typically includes high doses of corticosteroids for 4 to 8 weeks, followed by a gradual reduction in dosage. For persons requiring chronic or high doses of corticosteroids, or when improvement is not optimal, other treatments may be effective and are often recommended, including:
Methotrexate, azathioprine, or other immunosuppressive medications. These agents may reduce disease activity and allow reduction or even discontinuation of the corticosteroids.
Intravenous immunoglobulin (IVIG) - infusions of these proteins have been found to help many persons with dermatomyositis, but are generally reserved for those not responding to the other treatments.
Combinations of these treatments are occasionally used. For example, methotrexate and azathioprine are sometimes prescribed together to get muscle inflammation under control.
Non-steroidal anti-inflammatory drugs (NSAIDs), hydroxychloroquine or topical corticosteroid treatments may improve the joint pain and rash and allow reduction in the oral corticosteroid therap.
In up to 10 percent to 15 percent of cases, dermatomyositis may be associated with cancer. This is a particular concern in older persons, or when usual treatment is not effective. Some physicians recommend only the screening tests that are ordinarily appropriate for age and gender even if dermatomyositis is present, but others will also order CT or MRI scans of the chest, abdomen and pelvis looking for a tumor. The optimal approach is not clear, but your sister should review this possibility with her physicians if it has not already been addressed.
The specific problems your sister is having should determine which of these treatments might be best for her, or whether other evaluation might be in order.
(Robert H. Shmerling, M.D. is a practicing physician in rheumatology at Beth Israel Deaconess Medical Center, Boston, Mass., and an Associate Professor of Medicine at Harvard Medical School.)
(For additional consumer health information, please visit http://www.health.harvard.edu.)