McCann and Pain identified clinical findings that tend to be associated with a high risk of severe disease in patients with juvenile dermatomyositis (JDM). A patient with one or more of these findings may benefit from urgent referral to a specialist. The authors are from Alder Hey Children's NHS Foundation Trust in Liverpool.
Patient selection: juvenile dermatomyositis
Risk factors for severe disease:
(1) age less than 1 year
(2) severe disability (unable to get out of bed)
(3) severe weakness
(4) presence of aspiration or dysphagia and being unable to swallow
(5) skin ulcer
(6) need for management in the intensive care unit (ICU)
(7) major organ involvement (see below)
Major organ involvement:
(1) interstitial lung disease (ILD)
(2) gastrointestinal vasculitis with bleeding
(3) myocarditis
(4) decreased level of consciousness and/or seizures
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