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Clinical characteristics and outcomes of juvenile and adult dermatomyositis.

Na SJ, Kim SM, Sunwoo IN, Choi YC - J. Korean Med. Sci. (2009)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Konyang University College of Medicine, Daejeon, Korea.

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.

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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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Figure 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.

Mentions: The median follow-up duration of juvenile and adult DM patients was 52 months (range, 34-110 months) and 41 months (range, 3-110 months), and there was no statistically significant difference between two groups. The outcomes of patients with juvenile and adult DM are summarized in Table 3. In the 16 patients with juvenile DM, 9 patients (56.2%) had remission. Six patients (37.5%) had improvement of juvenile DM. The 1 remaining patient (6.3%) had a deteriorating course. None of the 11 patients with juvenile DM died during follow-up. Eighteen out of 48 adult DM patients (37.5%) had remission and 17 (35.4%) had improvement. Three patients (6.3%) had deterioration of adult DM. Ten out of the total 48 patients with adult DM died during the follow up. The mortality rate of adult DM was 20.8% and significantly higher than juvenile DM (0%) (P<0.001). The survival difference between juvenile and adult DM by log-rank test was statistically not significant (P=0.0544) (Fig. 1).


Clinical characteristics and outcomes of juvenile and adult dermatomyositis.

Na SJ, Kim SM, Sunwoo IN, Choi YC - J. Korean Med. Sci. (2009)

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.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2719214&req=5

Figure 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.
Mentions: The median follow-up duration of juvenile and adult DM patients was 52 months (range, 34-110 months) and 41 months (range, 3-110 months), and there was no statistically significant difference between two groups. The outcomes of patients with juvenile and adult DM are summarized in Table 3. In the 16 patients with juvenile DM, 9 patients (56.2%) had remission. Six patients (37.5%) had improvement of juvenile DM. The 1 remaining patient (6.3%) had a deteriorating course. None of the 11 patients with juvenile DM died during follow-up. Eighteen out of 48 adult DM patients (37.5%) had remission and 17 (35.4%) had improvement. Three patients (6.3%) had deterioration of adult DM. Ten out of the total 48 patients with adult DM died during the follow up. The mortality rate of adult DM was 20.8% and significantly higher than juvenile DM (0%) (P<0.001). The survival difference between juvenile and adult DM by log-rank test was statistically not significant (P=0.0544) (Fig. 1).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Konyang University College of Medicine, Daejeon, Korea.

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.

Show MeSH
Related in: MedlinePlus