1. Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis.
- Author
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Norimoto Kobayashi, Shunichiro Takezaki, Ichiro Kobayashi, Naomi Iwata, Masaaki Mori, Kazushige Nagai, Naoko Nakano, Mari Miyoshi, Noriko Kinjo, Takuji Murata, Kenji Masunaga, Hiroaki Umebayashi, Tomoyuki Imagawa, Kazunaga Agematsu, Shinji Sato, Masataka Kuwana, Masafumi Yamada, Shuji Takei, Shumpei Yokota, and Kenichi Koike
- Subjects
DERMATOMYOSITIS ,INTERSTITIAL lung diseases ,THERAPEUTIC use of biochemical markers ,ACADEMIC medical centers ,AUTOIMMUNE diseases ,BLOOD testing ,STATISTICAL correlation ,ENZYME-linked immunosorbent assay ,FERRITIN ,FISHER exact test ,IMMUNOGLOBULINS ,INTERLEUKINS ,PHYSICAL diagnosis ,RESEARCH funding ,STATISTICS ,DATA analysis ,RETROSPECTIVE studies ,DATA analysis software ,DESCRIPTIVE statistics ,CHILDREN ,DIAGNOSIS - Abstract
Objective. Rapidly progressive interstitial lung disease (RP-ILD) is a rare but potentially fatal complication of JDM. The aim of this study was to establish markers for the prediction and early diagnosis of RP-ILD associated with JDM. Methods. The clinical records of 54 patients with JDM were retrospectively reviewed: 10 had RP-ILD (7 died, 3 survived), 19 had chronic ILD and 24 were without ILD. Routine tests included a high-resolution CT (HRCT) scan of the chest and measurement of serum levels of creatine phosphokinase, ferritin and Krebs von den Lungen-6 (KL-6). Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies and IL-18 levels were measured by ELISA. Results. No differences were found in the ratio of juvenile clinically amyopathic DM between the three groups. Initial chest HRCT scan findings were variable and could not distinguish between RP-ILD and chronic ILD. Anti-MDA5 antibodies were positive in all 8 patients with RP-ILD and 10 of 14 with chronic ILD, but none of the patients without ILD. Serum levels of anti-MDA5 antibody, ferritin, KL-6 and IL-18 were significantly higher in the RP-ILD group than in the chronic ILD and non-ILD groups. Serum levels of IL-18 positively correlated with serum KL-6 (R = 0.66, P < 0.001). Conclusion. High serum levels of IL-18, KL-6, ferritin and anti-MDA5 antibodies (e.g. >200 units by ELISA) are associated with RP-ILD. These can be used as an indication for early intensive treatment. Both alveolar macrophages and autoimmunity to MDA5 are possibly involved in the development of RP-ILD associated with JDM. [ABSTRACT FROM AUTHOR]
- Published
- 2015
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