Once the response to treatment has been established the aim is slowly to decrease the steroids, but treatment may need to be quite prolonged – often for two years or more. Relapses may occur as treatment is being reduced or in association with chance infections.
Many children make a full recovery on steroid treatment alone. When relapses do occur it is often possible to bring the symptoms under control again by increasing or reintroducing the steroids. However, some children prove more resistant to, or are unable to tolerate steroids and in this situation the use of other drugs which suppress the body’s immune system is considered. These include azathioprine, methotrex, cyclophosphamide and cyclosporin and intravenous infusions of immunoglobulins.
Very rarely, children can become acutely and seriously ill and in this situation plasma exchange has been shown to produce a temporary improvement while the other drugs are beginning to work. Drugs such as azathioprine are much slower in their action than steroids and may take many weeks to produce the desired response.
Are there any side effects of treatment?
Unfortunately most drugs have some side effects, but left untreated juvenile dermatomyositis is a serious disease. Doctors try to minimise side effects of treatment as far as possible by using the lowest effective drug dosage and monitoring each child carefully as an outpatient. This will usually involve regular blood pressure measurements, urine and blood tests. Your doctor will explain the side effects of each drug and what to look out for. Most importantly, if you are unsure or unhappy about any part of your child’s treatment – do ask.
Can exercises help?
Once your child is beginning to respond to treatment active physiotherapy becomes an important part of therapy to help strengthen weak muscles and treat or prevent contractures, which lead to stiffness of joints. Contractures can occur early and compound the problem of muscle weakness. Regular physiotherapy will be started in hospital and arrangements will be made for the local services to take over when your child is well enough to go home. Your child will have a programme of exercises, which will be made as much fun as possible and you will be taught how to help with these at home. What about the future?
Most children make a full recovery from their disease. However the time taken may vary from a few months to several years in some cases. In a minority there may be some permanent weakness and contractures, although with the advent of cyclosporin therapy many such children have made significant progress. A rare complication of juvenile dermatomyositis is calcinosis – the formation of chalky material under the skin. This tends to occur when the muscle inflammation has settled and may improve only very slowly. In some instances surgical removal of the deposits may be necessary. All children should be encouraged to be as active as possible except during the acute disease and, above all, parents should be optimistic about the future.