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Progression
The speed of the progression of JDM is highly variable. Nearly all JDM patients
have some skin involvement. The JDM rash usually occurs as the initial symptom.
Sometimes it is so slight as not to be recognized for what it is until muscle
symptoms appear. Sometimes muscle symptoms never appearing at all or occur very
gradually over the course of months, and sometimes going from normal strength to
being unable to walk within days. Usually, muscle symptoms appear weeks to
months after the onset of the rash.
Diagnosis
JDM is diagnosed by a combination of patient/parent observations, clinical
examination, and laboratory blood tests.
Treatment
Once a diagnosis of JDM is made, the treatment is often a 3-day course of
Intravenous ("pulse") steroids (methylprednisolone, Solu-Medrol®), followed by a
high dose of oral prednisone (usually 1-2mg/kg of body weight) for several
weeks. This action usually brings the disease under control, lowering most lab
tests to or near normal values. Some minor improvement in muscle symptoms may
also be seen in this time, but normally it takes a long time for full muscle
strength to be regained.
Once the disease process is under control, oral steroids are tapered gradually
to minimize their side effects. Sudden withdrawal of oral steroids is dangerous.
Often, steroid-sparing drugs, such as methotrexate (a chemotherapy drug in much
higher doses than is given for JDM) or other DMARDs, are given to compensate for
the reduction in oral steroids. Once the oral steroids are reduced to a less
toxic level, the sparing agents can also be gradually withdrawn. Lab results are
closely monitored during the tapering process to ensure that the disease does
not recur.
In the cases where steroids or second-line drugs are not tolerated or are
ineffective, there are other treatments that can be tried. These include other
chemotherapy drugs, such as ciclosporin, infliximab, or other DMARDs. Another is
intravenous immunoglobulin (IVIg), a blood product that has been shown to be
very effective against JDM.
To treat the skin rash, anti-malarial drugs, such as hydroxychloroquine (Plaquenil®)
are usually given. Topical steroid creams (hydrocortisone) may help some
patients, and anti-inflammatory creams (such as tacrolimus) are proving to be
very effective. Dry skin caused by the rash can be combated by regular
application of sunscreen or any moisturizing cream, such as Eucerin. Most JDM
patients are very sensitive to sun exposure, and sunburn may be a disease
activity trigger in some, so daily application of high-SPF sunscreen is often
recommended.
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