Juvenile dermatomyositis
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Juvenile dermatomyositis | |
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Juvenile dermatomyositis | |
Specialty | Rheumatology |
Juvenile dermatomyositis (JDM) is an
Signs and symptoms
The vasculitis caused by JDMS manifests itself predominantly in two ways:
One is a distinctive
The second symptom caused by vasculitis is
Other symptoms may include irritability, weight loss, and mouth ulcers.[2] When a child becomes irritable, fatigued, reluctant to socialize, and the face becomes flushed easily, physicians refer to this constellation of symptoms as "misery."[3]
Progression
The speed of the progression of JDMS is highly variable. Nearly all JDM patients have some skin involvement. The JDMS 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 appear 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.[citation needed]
Cause
The underlying cause of JDM is unknown. It most likely has a genetic component, as other autoimmune disease tend to run in the families of patients. The disease is usually triggered by a condition that causes immune system activity that does not stop as it should, but the trigger is almost certainly not the cause in most cases. Common triggers include immunizations, infections, injuries, and sunburn.[citation needed]
Diagnosis
Proximal muscle weakness, characteristic skin rash and elevated muscle enzymes are routinely used to identify JDM. Typical magnetic resonance imaging and muscle biopsy changes are considered the next most useful diagnostic criteria, followed by myopathic changes on electromyogram, calcinosis, dysphonia and nailfold capillaroscopy. Other useful criteria include myositis-specific or -related antibodies, nailfold capillaroscopy, factor VIII-related antigen, muscle ultrasound, calcinosis and neopterin.[4]
Treatment
Once a diagnosis of JDMS 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 1–2 mg/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.[citation needed]
Once the disease process is under control, oral steroids are tapered gradually to minimize their side effects. Often, steroid-sparing drugs, such as
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
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 combatted by regular application of sunscreen or any moisturizing cream. 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.[citation needed]
Prognosis
Of the children diagnosed with and treated for JDM, about half will recover completely. Close to 30 percent will have weakness after the disease resolves.[6] Most children will go into remission and have their medications eliminated within two years, while others may take longer to respond or have more severe symptoms that take longer to clear up.[7]
A common lasting effect of JDM is childhood arthritis.[citation needed]
Gallery
References
- ^ [1] Archived July 5, 2008, at the Wayback Machine
- ^ "Juvenile Dermatomyositis | Boston Children's Hospital". Childrenshospital.org. Retrieved 2015-09-27.
- S2CID 37761391.
- PMID 16467366.
- S2CID 205951454.
- ^ "Dermatomyositis: Outlook". Freemd.com. 2010-12-09. Retrieved 2015-09-27.
- ^ "Juvenile Dermatomyositis (JDM)". Cincinnatichildrens.org. Retrieved 2015-09-27.