Medical Management Dermatomyositis

Medical Management

Dermatomysitis (DM) is a highly treatable disease. Some people, especially children, recover completely, while others experience greatly diminished symptoms for long periods of time. Several years of treatment to suppress the immune system may be necessary to achieve these results.

Those who don’t recover completely may need to continue on at least a low dose of medication to control the autoimmune attack of DM throughout their lives.

Drugs that suppress the immune system are the mainstay of therapy. The chart below describes the common treatments for DM and how they work. Treatment is similar to that for the muscle disease polymyositis (PM).

Avoidance of sun exposure during peak hours and use of sunblock and protective clothing are recommended to avoid exacerbating the skin aspects of the disease.

Children with DM are treated with the same medications and therapies as adults. They 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.

Permanent loss of strength and muscle atrophy sometimes occurs in DM, but in other cases, full strength and muscle size are recovered.

Medication or treatment	How it works	Comments
corticosteroids prednisone tablets (Deltasone); intravenous methylprednisolone sodium succinate (Solu-Medrol)	Dampens inflammation and immune response by interfering with processing of antigens and with early triggering of T cell and B cell production and later proliferation of B cells and T cells. These cells are produced by the immune system in autoimmune diseases such as PM and DM.	Can be taken orally as prednisone and related compounds; also available for intravenous use. Many side effects with long-term, high-dose therapy, such as weight gain and redistribution of fat to face, abdomen and upper back; thinning of skin; susceptibility to infection; bone loss; muscle damage; cataracts; elevated pressures in eyes (glaucoma); psychological disturbances; high blood pressure; high blood sugar; growth slowing in children.
azathioprine (Imuran)	Interferes with proliferation of B cells and T cells.	Can suppress production of several types of blood cells, so cell counts must be monitored; increases risk of cancer.
methotrexate (Rheumatrex, Folex, Mexate)	Interferes with proliferation of B cells and T cells.	Can cause liver damage; used in higher doses to treat cancer.
cyclosporine (Neoral, Sandimmune)	Keeps T cells from stimulating production of more T cells and B cells (“upstream” of azathioprine and methotrexate action).	Doesn’t affect production of cells other than T cells and B cells; can cause kidney damage, infection, high blood pressure, tremor and excessive hair growth.
cyclophosphamide (Cytoxan)	Interferes with proliferation and activity of B cells and T cells	Also used in cancer; toxic to many kinds of cells, including those of the blood and bladder; can cause sterility in both sexes.
mycophenolate mofetil (CellCept)	Interferes with proliferation of B cells and T cells.	Can cause diarrhea, vomiting, infection (particularly with cytomegalovirus); increases risk of cancer, especially lymphomas; causes depletion of certain blood cells.
tacrolimus (Prograf, old name FK506)	Keeps T cells from stimulating production of more T cells and B cells (“upstream” of azathioprine and methotrexate action).	Can damage kidneys; can cause headaches, tremors and sleep difficulties; diarrhea, nausea and vomiting; high blood pressure, high blood sugar and high blood levels of potassium; increases risk of infection and lymphomas. Drug breakdown interfered with by grapefruit juice; potential for kidney damage increased by some anti-inflammatory drugs.
hydroxychloroquine sulfate (Plaquenil)	Mechanism not understood; used in arthritis, lupus, malaria; can be used to reduce steroid dosage in myositis, particularly in children.	Can treat muscle symptoms and dermatomyositis rash. Can cause damage to eyes’ retinas or corneas; regular eye exams needed.
infusion of mixed immunoglobulins; IVIg (Gammar, Gammagard, Sandoglobulin, others)	Has complex actions on immune system, such as providing antibodies against patient’s own antibodies; interfering with immune system reaction to antibody-marked cells; interfering with blood-transported chemicals released by immune system; interfering with activation and maturation of T cells and B cells.	Doesn’t affect production of cells other than T cells and B cells; can cause kidney damage, infection, high blood pressure, tremor and excessive hair growth.
plasmapheresis	Removes antibodies and proteins made by the immune system from the blood and returns “cleansed” blood to patient.	Very rarely used in myositis since 1992 study showed it was no more effective than placebo; some think it’s useful when combined with immunosuppressant drugs.

Disease:

Dermatomyositis (DM)

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