J Korean Med Sci.  2009 Aug;24(4):715-721. 10.3346/jkms.2009.24.4.715.

Clinical Characteristics and Outcomes of Juvenile and Adult Dermatomyositis

• Na SJ
• Kim SM
• Sunwoo IN
• Choi YC

• KMID: 1779209
• DOI: http://doi.org/10.3346/jkms.2009.24.4.715

Abstract

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.

Keyword

Juvenile Dermatomyositis; Adult Dermatomyositis; Clinical Characteristics; Outcomes

MeSH Terms

Adolescent
Adult
Age of Onset
Aged
Anti-Inflammatory Agents/therapeutic use
Calcification, Physiologic
Dermatomyositis/*diagnosis/mortality/therapy
Exanthema/diagnosis
Female
Humans
Male
Middle Aged
Muscle Weakness/diagnosis
Prednisolone/therapeutic use
Prognosis
Severity of Illness Index
Survival Rate

Figure

• 

  Fig. 1 Survival curve of 16 patients with juvenile DM and 48 with adult DM. Number of available adult DM patients: 42 at 1-yr follow up, 40 at 3-yr follow up, and 38 at 9-yr follow up.

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