Characteristics of juvenile dermatomyositis in Japan

Kobayashi, Shinichi; Higuchi, Kenji; Tamaki, Hisashi; Wada, Yasuyuki; Wada, Noriyuki; Kubo, Masakatsu; Koike, Yuichi; Nagata, Michio; Tatsuzawa, Osamu; Fujikawa, Satoshi

doi:10.1111/j.1442-200x.1997.tb03595.x

Cited by 24 publications

(14 citation statements)

References 10 publications

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“…We also found that calcinosis and pain were related to higher CHAQ scores. Again, this result is not surprising, and is consistent with data reported by other investigators (12)(13)(14).…”

Section: Discussionsupporting

confidence: 93%

Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis

Huber

Lang

LeBlanc

et al. 2000

Arthritis & Rheumatism

241

144

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Objective. To evaluate functional outcomes in a cohort of patients with juvenile dermatomyositis (DM).Methods. A retrospective inception cohort of patients diagnosed as having juvenile DM between January 1, 1984 and January 1, 1995 was established at 4 Canadian tertiary care pediatric centers. Informed consent was obtained. Each subject was interviewed by telephone or in person. The primary outcome was physical function, as measured by the Childhood Health Assessment Questionnaire (CHAQ). Additional outcomes were educational and vocational achievement, growth, development of calcinosis, patient satisfaction with outcome, and development of other illnesses. Data regarding illness presentation, treatment, and disease course were obtained through chart review.Results. Sixty-five of 80 patients (81%; 46 females and 19 males) could be contacted. The median followup time was 7.2 years (range 3.2-13.9 years), with a median age at followup of 13 years (range 7-26 years). Twentyfour patients (37%) had a monocyclic course, while the remaining 41 (63%) had a chronic continuous or polycyclic course. Sixty-two patients (95%) were treated with corticosteroids, while 41 (63%) received a second-line agent. Physical function was excellent, with a median CHAQ score of 0 (range 0-2.50). Eighteen patients had scores >0, and only 5 had moderate-to-severe disability, as defined by a CHAQ score >1.0. Females had higher CHAQ scores, and all but 1 of the patients with scores >0 were female (range 0-2.50; P ‫؍‬ 0.015). Patients with a chronic continuous course also had higher CHAQ scores. Sixteen patients in the chronic continuous group had CHAQ scores >0 (range 0-2.50; P ‫؍‬ 0.0009). Calcinosis developed in 22 patients (34%) and persisted to followup in 14. Development of calcinosis was not related to initial therapy, sex, or disease course, but was significantly associated with higher CHAQ scores (range 0-1.0 versus 0-2.5; P ‫؍‬ 0.01). At the time of followup, 26 patients (40%) still had rash, 15 (23%) still reported weakness, and 23 (35%) continued taking medications, despite the fact that all were at least 3 years postdiagnosis. There was 1 death.Conclusion. In general, patients in this cohort had favorable outcomes. Most had CHAQ scores of 0, and only 8% met our definition of moderate-to-severe disability. However, many patients continued to have chronic disease, persistent rash, and continued taking medications >3 years after diagnosis. Further research is needed to improve outcomes for patients with juvenile DM.Juvenile dermatomyositis (DM) is a chronic inflammatory disorder of unknown etiology that affects primarily muscle and skin. It has an incidence of ϳ2-3/ 1,000,000/year. Prior to 1960, outcomes were poor, with up to one-third of patients dying of their illness, onethird developing permanent, severe physical limitations, and one-third recovering completely (1). Reports published through the 1970s and 1980s have shown a marked decrease in mortality (ϳ10%) and have suggested an improvement in functional outcomes (2-5).

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“…We also found that calcinosis and pain were related to higher CHAQ scores. Again, this result is not surprising, and is consistent with data reported by other investigators (12)(13)(14).…”

Section: Discussionsupporting

confidence: 93%

Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis

Huber

Lang

LeBlanc

et al. 2000

Arthritis & Rheumatism

241

144

View full text Add to dashboard Cite

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“…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.…”

Section: Discussioncontrasting

confidence: 56%

“…The positive rates for EMG and muscle biopsy in JDM with muscular signs have been reported to be 60%-100% and 90%-100%, respectively, 9,11 and they have thus been established as a valuable tool for the diagnosis of JDM. 15,16 In recent MRI studies, fat-suppressed MRI made it easy to identify the muscular, cutaneous, subcutaneous, and fascial changes in the early disease course.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2007

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To clarify the clinical features of juvenile dermatomyositis (JDM) in Japanese cases, we retrospectively evaluated the characteristics of 16 children with JDM that were treated at Saitama Children's Medical Center between 1985 and 2004. The age at disease onset ranged from 3.5 to 14.1 years old (7 boys, mean age 7.9 years; 9 girls, mean age 9.2 years). In 14 patients more than two muscle enzymes were elevated at diagnosis. The antinuclear antibody at diagnosis was positive in all girls but one, while it was positive in only two boys (2/7; P<0.01). Three patients were complicated with interstitial lung disease (ILD) (18.8%) and their serum KL-6 levels were already elevated on admission. Our findings suggest that serum KL-6 levels seemed to be sensitive to the detection of ILD in an early phase, and the relatively high frequency of JDM-associated ILD indicated that a careful evaluation of the lungs was therefore required in any individuals with JDM. Of 16 patients, two boys showed a favorable improvement and prognosis without relapse for over 9 years after the termination of treatment. Overall, in girls, there is a tendency to be a delay in the diagnosis/treatment for JDM, and this disease also demonstrated a severe course.

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“…ILD occurs after the diagnosis of polymyositis or dermatomyositis in up to 40% of patients, and precedes the diagnosis of overt CTD in 20-30% of cases [47,52]. In large cohorts of JDM/JPM, ILD occurs in 8-13% of patients and thus seems to occur less frequently than in adult dermatomyositis [53,54]. Risk factors for ILD in a patient with dermatomyositis/polymyositis include genetic predisposition [55,56], some clinical signs such as arthralgia [47], and autoimmune features (table 2) [22,56,57].…”

Section: Direct Pulmonary Involvementmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

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Idiopathic inflammatory myositis (IIM) is a group of rare connective tissue diseases (CTDs) characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD) is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD. @ERSpublications Interstitial lung disease is a leading cause of morbidity in dermatomyositis/polymyositis

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Characteristics of juvenile dermatomyositis in Japan

Cited by 24 publications

References 10 publications

Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis

Medium- and long-term functional outcomes in a multicenter cohort of children with juvenile dermatomyositis

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

Idiopathic inflammatory myopathies and the lung

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