Open AccessClinical Characteristics and Outcomes of Juvenile and Adult Dermatomyositis
Author(s) -
Sang-Jun Na,
Seung Min Kim,
Il Nam Sunwoo,
Young Chul Choi
Publication year - 2009
Publication title -
journal of korean medical science/journal of korean medical science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.743
H-Index - 66
eISSN - 1598-6357
pISSN - 1011-8934
DOI - 10.3346/jkms.2009.24.4.715
Subject(s) - juvenile dermatomyositis , medicine , dermatomyositis , juvenile , rash , dysphagia , proximal muscle weakness , connective tissue , pathology , connective tissue disease , age of onset , malignancy , subcutaneous tissue , muscle biopsy , disease , biopsy , surgery , autoimmune disease , biology , genetics
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.