Clinical characteristics and outcomes of juvenile and adult dermatomyositis.
Dermatomyositis (DM) is an idiopathic inflammatory myopathy with bimodal onset age distribution. The age of onset is between 5-18 yr in juvenile DM and 45-64 yr in adult DM. DM has a distinct clinical manifestation characterized by proximal muscle weakness, skin rash, extramuscular manifestations (joint contracture, dysphagia, cardiac disturbances, pulmonary symptoms, subcutaneous calcifications), and associated disorders (connective tissue disease, systemic autoimmune diseases, malignancy). The pathogenesis of juvenile and adult DM is presumably similar but there are important differences in some of the clinical manifestations, associated disorders, and outcomes. In this study, we investigated the clinical characteristics and outcomes of 16 patients with juvenile DM and 48 with adult DM. This study recognizes distinctive characteristics of juvenile DM such as higher frequency of neck muscle involvement, subcutaneous calcifications, and better outcomes.
Na
Sang-Jun
SJ
Department of Neurology, Konyang University College of Medicine, Daejeon, Korea.
Kim
Seung Min
SM
Sunwoo
Il Nam
IN
Choi
Young-Chul
YC
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002113:Calcification, Physiologic; D003882:Dermatomyositis / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D005076:Exanthema / Q000175:diagnosis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000175:diagnosis; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D012720:Severity of Illness Index; D015996:Survival Rate
D000893:Anti-Inflammatory Agents; D011239:Prednisolone