Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Juvenile dermatomyositis is part of the idiopathic inflammatory myositis group of diseases, presenting acute or chronic multisystemic inflammatory injury, that mainly affects muscles and skin. It s incidence is 4/1,000,000, prevailing in women and Caucasians, with symptoms starting between 5 14 years of age. The etiology is unknown, nevertheless is well recognized the role of vasculitis produced by autoantibodies, such is responsible for the damage on skin and muscular microvasculature. The acute or chronic complications and the neuropathic manifestations (cerebral vasculitis and peripheric neuropathy) are rare, and both are the result of the same autoimmune vasculitis injury.
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PMID:[Juvenile dermatomyositis]. 1198 14

Here we report two patients with severe juvenile dermatomyositis (JDM) complicated with extra musculocutaneous involvement. The first case (a 10-year-old boy) had unusual initial presentation of JDM complicated with interstitial lung disease documented with high-resolution computed tomography. He had a rapidly progressive course and died in 7 weeks after the onset of the disease despite steroid and immunosuppressive treatment. The second case (a 14-year-old boy) was presented with myositis complicated with hepatitis. He also had a chronic course of JDM with unfavorable outcome. It appears that the prognosis of patients with severe JDM is related with the degree of autoimmune vasculitis on extra musculocutaneous involvement.
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PMID:Severe juvenile dermatomyositis: two patients complicated with extra musculocutaneous involvement. 1672 99