Treatment

Treatment for juvenile dermatomyositis uses a multidisciplinary approach and studies have shown that the earlier treatment starts after disease onset the better. The dilemma of treating patients with JDM is judging the severity of disease as under-treatment can lead to long term disability. The principle behind current treatments is to induce symptom remission as rapidly as possible. Once the diagnostic investigations have been performed and juvenile dermatomyositis has been confirmed, treatment can proceed.

It is very important that the prescribed medicines are taken regularly. Medicines for JDM work in the background over time. So although your child may not notice a difference if one dose is missed, the medicines will not work as well if several doses are missed. All the medicines used to treat JDM decrease activity of the immune system. As a group, they are called ‘immunosuppressants.’ Each drug suppresses the immune system to a greater or lesser degree. Therefore, your child may be more at risk of infection and you should seek medical advice if your child becomes unwell. Some vaccinations cannot be given whilst on these medicines.

The first line of treatment is the use of Corticosteroids; these can be given intravenously (into a vein) or by mouth. Disease-modifying anti-rheumatic drugs (DMARDS) are added to control the disease long-term, as evidence suggests better disease control is achieved when these drugs are used in conjunction with corticosteroids. Supplements such as Folic acid, Calcium, and Vitamin D may also be prescribed.

Here are some of the drugs that may be used. NB: This is not a complete description of the drugs and side effects and your child should be given information by your specialist doctor or nurse.

Steroids

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Prednisolone
  • This is the most effective drug for controlling JDM initially because it works quickly. This drug works to decrease the inflammation caused by JDM. Side effects of prednisolone are related to the dose taken and how long it is taken. When your child is diagnosed a high dose will usually be required. As your child’s JDM improves, the dose will be lowered gradually and any side effects experienced will get less and disappear. Side effects that may be noticed include an increase in appetite, an increase in weight, a “puffy face,” acne, mood swings, stretch marks, increased risk of infection, slower growth, and increased body hair. In the long term, your child may develop weaker bones (but there are now treatments to prevent this), high blood pressure, or cataracts (a cloudy area that develops in the lens of the eye. These are different from cataracts seen in older people and usually do not affect vision). You may feel anxious about your child taking prednisolone after reading the above side effects; however, it is the best drug initially for treating JDM initially. Doctors would not prescribe it if the illness could be treated in a better way. Side effects are dose-related (more likely to occur if higher doses are given for a long time) and doctors will always use the lowest possible dose to help your child get better. It is very important to wait until the doctor guides your child about reducing the steroid dose. Prednisolone should NEVER be stopped suddenly. This may make your child extremely sick. The doctor will reduce your child’s prednisolone gradually guided by symptoms present and blood tests.
Methylprednisolone
  • This drug is similar to prednisolone except it is given into a vein in your child’s arm. It is often given in high doses and in the early stages of JDM when it is more active or may be given in times of disease flare (worsening).

Disease Modifying Anti-Rheumatic Drugs (DMARDS)

Treatment with steroids is usually associated with the use of other drugs that treat JDM by suppressing (decreasing) the immune system. These drugs are called ‘Disease Modifying Drugs’. The most common drug used in addition to steroids is methotrexate. Although this works very well in 70-80% of children with JDM, some children will need alternative medicines. Different DMARDS are used in severe diseases from those used in mild diseases. 

Methotrexate and many other DMARDS take at least 6-8 weeks to start to take effect and so steroids need to be used first to control JDM. All DMARDS work by suppressing the immune system in some way, and so your child will be more prone to infections. This is not usually too much of a problem, but you need to seek medical attention sooner than you would if your child were not taking these medicines. Some vaccinations cannot be given whilst taking these drugs. You may need to stop your child’s DMARD medicine (but not steroids) temporarily if your child is unwell with a temperature. You should seek advice from your doctor or nurse specialist if you are unsure what to do.

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Methotrexate
  • This drug reduces inflammation (redness, pain, swelling) by acting directly on the immune system. It is usually given over a longer period of time and helps to maintain remission (keeping the disease under control) when reducing the steroid dose. Methotrexate can affect the white blood cells, which fight infection, and also the liver enzymes, so regular blood tests are required.  Methotrexate has been used for many years and tends to be the first treatment given for most patients with JDM in addition to steroids.

Other medicines that may need to be used instead of methotrexate or in addition to methotrexate

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Azathioprine
  • This drug helps to reduce inflammation by suppressing the immune system and like other DMARDS, regular blood tests are needed.  It can sometimes cause sickness or rashes.
Mycophenolate Mofetil (MMF)
  • This drug also suppresses the immune system and therefore regular blood tests are required. MMF can cause diarrhoea when it is first given. For this reason, it is usually started in a low dose and increased over a few weeks.
Ciclosporin
  • Like methotrexate, this is usually given over a longer period of time. Long-term side effects include raised blood pressure, increase in body hair, gum enlargement, and kidney problems.
Hydroxychloroquine
  • This is a milder medication that can be given in addition to drugs such as methotrexate. It can be particularly good at helping skin disease or joint aches and pains. If your child is taking hydroxychloroquine, they should have an eye test with an optician once a year since it can (rarely) cause changes in colour vision or peripheral vision (seeing objects outside the direct line of vision). This usually recovers once the drug is stopped
Immunoglobulins (IVIG)
  • This product contains human antibodies (proteins) concentrated from blood donors. It is given through a vein in your child’s arm. IVIG works through the immune system, helping the inflammation. It is usually given in addition to other medicines such as methotrexate and can be useful for skin disease or generally decreasing inflammation to allow steroid doses to be reduced.
Cyclophosphamide
  • This is a more powerful immunosuppressive drug that helps decrease the activity of cells causing inflammation. It is usually given once a month through a vein in your child’s arm and requires an overnight stay in hospital. Cyclophosphamide is usually only given in more severe cases of disease and is needed when there is organ involvement (eg. lungs, brain) or severe skin or muscle changes.

Biologic Agents

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Infliximab
  • Infliximab is a type of medication called a biologic that targets the immune system in a more specific way. It is given through a vein in your child’s arm. Like most of the other medicines, this works by reducing inflammation and regular blood tests are required whilst on infliximab. It tends to be given as an extra treatment in those children that do not respond well enough to initial medication with drugs such as methotrexate.
Rituximab
  • This is also an immunosuppressive drug used in severe disease and usually is given after other drugs have been tried. It is given through a vein in your child’s arm. Two doses are usually given two weeks apart and then may need to be repeated at a later date.
Adalimumab
  • Adalimumab (Humira) is a biologic agent used for the treatment of inflammatory myopathies. It reduces inflammation by inhibiting the activity of a chemical ‘cytokine’ in the body called ‘tumour necrosis factor alpha’ (TNF-alpha). It may be given in children with active disease that do not respond to standard treatments or if they are intolerant to or have medical reasons that should not take such treatments. It is given as a subcutaneous injection as methotrexate once every two weeks.
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Office Location

Juvenile Dermatomyositis Cohort Biomarker Study & Repository (JDCBS)
UCL Great Ormond Street
Institute of Child Health
6th Floor
30 Guildford Street
London, WC1N 1EH