Dermatomyositis

Medical Management

Dermatomyositis is a highly treatable disease. Drugs that suppress the immune system (immunosuppressive drugs) are the mainstay of therapy.

Immunosuppressive therapy

Glucocorticoids, particularly prednisone, are often the first-line treatment. These medications help to reduce inflammation and swelling, and suppress damaging autoimmune responses. Long-term use of high-dose glucocorticoid therapy, however, may produce unwanted side effects, such as decreased bone density, increased tissue swelling (edema) and/or weight gain.

For some affected people, therapy with other immunosuppressive drugs, such as azathioprine, methotrexate, mycophenoloate mofetil, tacrolimus, or cyclosporine may be beneficial. Intravenous immunoglobulin (IVIG) has been recently approved by the FDA for the treatment of dermatomyositis in July 2021. Additional treatment options used in combination may include the monoclonal antibody rituximab. The antimalarial agent hydroxychloroquine may also be used for mild skin predominant dermatomyositis.

Treatment plans are typically individualized to meet the needs of the affected person and ongoing monitoring is required to optimize the treatment protocol. In some cases, affected people may require prolonged maintenance therapy throughout their lives, while in others, therapy may be gradually withdrawn as symptoms resolve. Several years of treatment to suppress the immune system may be necessary to achieve these results.

Supportive care

Multidisciplinary care can improve the overall health of people living with dermatomyositis, and may include recommendations from a variety of specialists. Surgical removal of calcium deposits is sometimes recommended to treat painful calcinosis. Physical therapy may be recommended to help improve muscle strength and avoid development of contractures (muscle shortening/tightening). Supportive treatment can help manage complications such as swallowing or breathing difficulties or abnormalities of the heart, lungs, or gastrointestinal tract. In people with associated malignancy, removal of the cancer may be required. Non-pharmacological treatments to avoid exacerbating the skin aspects of dermatomyositis may include sun avoidance and use of sunscreens and protective clothing.

Juvenile dermatomyositis (JDM)

Children with juvenile dermatomyositis (JDM) are treated with immunosuppressive medications
such as steroids, methotrexate, and IVIG. While some treatment options may be similar
between adults and juveniles with dermatomyositis, depending on the organ involvement,
different immunosuppressants such as cyclosporin and TNF inhibitors may be tried. There are
also reports that JAK inhibitors may be beneficial in juveniles, similar to the treatment landscape in adults. Children with JDM may have to be kept out of physical education classes during
periods of acute disease activity.

Myositis and You: A Guide to Juvenile Dermatomyositis for Patients, Families and Healthcare Providers, is a 480-page book written by experts in this disease and the grandmother of a child affected by it. Published in 2007, it’s available at bookstores and through the Internet.

References

1. Dalakas MC. Inflammatory Muscle Diseases. Longo DL, ed. N Engl J Med. 2015;372(18):1734-1747. doi:10.1056/NEJMra1402225
2. Baig S, Paik JJ. Inflammatory muscle disease – An update. Best Pract Res Clin Rheumatol. 2020;34(1):101484. doi:10.1016/j.berh.2019.101484

Last update: Feb 2023
Reviewed by Julie Paik, MD, MHS; Johns Hopkins University