Juvenile Dermatomyositis: A Statistical Study of 114 Patients with Dermatomyositis

Abstract

We conducted a statistical review of 114 cases of dermatomyositis (DMS) treated primarily at the Department of Dermatology at Nagoya University Hospital over 27 years from 1965 to 1991 in order to determine the primary characteristics of juvenile DMS with the following results. 1) Juvenile DMS was found slightly more often in males than females; the male-to-female ratio was 1.4:1. Therefore, unlike adult DMS with its preponderance of females, there was no clear gender predominance. 2) Muscular manifestations tended to follow the appearance of cutaneous manifestations, but the frequency of minor muscular manifestations was high over the entire course of the disease. 3) Laboratory findings showed increases in serum aldolase and serum creatinine kinase with significant frequency when compared with adult patients (p < 0.01 and p < 0.05, respectively). Elevated serum aldolase most often occurred prior to or at the time of the appearance of muscular manifestations, suggesting its usefulness in early diagnosis. The positive rates for the antinuclear antibody on HEp-2 cells and anti-DNA antibody were significantly lower in children than in adults (p < 0.001 and p < 0.05, respectively). 4) There were no cases of juvenile DMS complicated by malignant tumors, interstitial pneumonia, or pulmonary fibrosis. There were also no deaths, and the rate of "remission or improvement" was significantly higher than in adult DMS cases (p < 0.05). Adult cases which remained the same or worsened usually presented with intractable muscular manifestations. In children, however, the cutaneous manifestations were more difficult to treat.(ABSTRACT TRUNCATED AT 250 WORDS)

Clinical Characteristics and Outcomes of Juvenile and Adult Dermatomyositis

“…In our study, onset age distribution was similar to that of previous reports on juvenile DM (2, 12), but there was a wider variation of onset age distribution in adult DM. It is reported that females outnumber males by 2: 1 (10, 11), and our study also showed female predominance in both juvenile and adult DM.…”

Juvenile dermatomyositis: clinical characteristics and the relatively high risk of interstitial lung disease

“…The frequency of ILD in JDM has been reported to be low in comparison with that in adult DM. [9][10][11] However, the report by Kobayashi et al 6 indicated that the frequency of ILD seems to be high in our study (Table 3). These results might be associated with the severity of the disease, and recent advances in such diagnostic modalities as CT scans and serum KL-6, or Japanese ethnogenesis, e.g., human leukocyte antigen molecules and genes.…”

“…10 The positivity for the antinuclear antibody in our cases was more frequent than that described in previous reports, with an incidence 15%-23%, thus indicating a similarity in adult DM. 9,13 In our study the antinuclear antibody was not considered to be a helpful marker of the disease severity or activity.…”

“…12 Creatine kinase and aldolase are effective for evaluating the treatment of the disease in adult DM and the abnormalities of what are more incidental to JDM than adult DM. 4,9,13,14 Of 4 patients (3 boys and 1 girl) with maximum CK levels >7000 IU/l and extremely high levels of aldolase and myoglobin, 3 boys had a favorable clinical course, thus suggesting that the earlier diagnosis (<6 months) and therapy initiation contributed to their good outcome. In their clinical course, CK levels almost paralleled aldolase.…”

Juvenile idiopatiske inflammatoriske myopatier

“…Studier fra Japan og Saudi-Arabia har derimot vist en overhyppighet hos gutter, med en jente: gutt ratio på 1: 1,3-1,5 (18,19). Hos barn under 2 år er sykdommen like vanlig hos begge kjønn (11,12,20).…”