Background Juvenile dermatomyositis (JDM) display differing prevalence of features in comparison with adult-onset dermatomyositis. Because of a few numbers of reports about interstitial lung disease (ILD) in JDM, little is known about the pathogenesis and prognostic factors of ILD in JDM.
Objectives The aim of this study is to evaluate the clinical manifestiation and prognostic factors of ILD with JDM.
Methods We retrospectively reviewed the clinical information, laboratory data of severely ill cases who were diagnosed as defined and probable JDM in Japan. The records of 25 patients with ILD were identified. Six cases of them died and 19 patients were alive. ILD is the major cause of death in Japanse JDM patients. Clinical manifestiations and serum IL-18, BAFF and IFNa levels of dead patients were compared with those in alive patients.
Results All dead cases had acute or subacute ILD and showed mild grade of skin and muscle disorder in compaered with the recovery group. Serum AST, ALT and KL-6 were higher and CK level was lower than those of the recovery cases. The dead cases tend to have the high levels of serum BAFF and IL-18, however, there are no correlation of severity of ILD in ferritin values and serum IFNa levels. Only one case had positive anti-Jo-1 antibody. In almost cases of JDM with ILD, anti-CADM-140/MDA5 antibody is positive and dead cases had a tendency with higher levels of that antibody. The lung pathology of the examples from 4 dead cases is diffuse alveolar damage, suggested existence of prominent blood vessel inflammation.
Conclusions This study underlines that ILD results in high morbidity in JDM.
Disclosure of Interest N. Kobayashi Grant/Research support from: Health and Labour Science Research Grant, S. Sato: None Declared, M. Mori Grant/Research support from: Health and Labour Science Research Grant, K. Koike: None Declared, S. Yokota Grant/Research support from: Health and Labour Science Research Grant
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