title: Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis.
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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Norimoto
authors.initials: N
authors.lastname: Kobayashi
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Shunichiro
authors.initials: S
authors.lastname: Takezaki
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan ichikobaya@med.hokudai.ac.jp.
authors.forename: Ichiro
authors.initials: I
authors.lastname: Kobayashi
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Naomi
authors.initials: N
authors.lastname: Iwata
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Masaaki
authors.initials: M
authors.lastname: Mori
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Kazushige
authors.initials: K
authors.lastname: Nagai
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Naoko
authors.initials: N
authors.lastname: Nakano
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Mari
authors.initials: M
authors.lastname: Miyoshi
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Kinjo
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Takuji
authors.initials: T
authors.lastname: Murata
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Masunaga
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Umebayashi
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Imagawa
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Kazunaga
authors.initials: K
authors.lastname: Agematsu
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Sato
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Masataka
authors.initials: M
authors.lastname: Kuwana
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Masafumi
authors.initials: M
authors.lastname: Yamada
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Shuji
authors.initials: S
authors.lastname: Takei
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Shumpei
authors.initials: S
authors.lastname: Yokota
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Ohbu, Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Department of Pediatrics, Ehime University Graduate School of Medicine, Matsuyama, Department of Allergy & Immunology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Department of Pediatrics, University of the Ryukyus, Naha, Department of Pediatrics, Osaka Medical College, Takatsuki, Department of Pediatrics, Kurume University School of Medicine, Kurume, Department of General Pediatrics, Miyagi Children's Hospital, Sendai, Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center, Yokohama, Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, Matsumoto, Department of Rheumatology, Tokai University School of Medicine, Isehara, Department of Rheumatology, Keio University School of Medicine, Tokyo and School of Health Science, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Ariga
mesh_terms: D000293:Adolescent; D000888:Antibodies, Anti-Idiotypic / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D053487:DEAD-box RNA Helicases / Q000276:immunology; D003882:Dermatomyositis / Q000097:blood* / Q000150:complications* / Q000208:ethnology; D018450:Disease Progression; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007223:Infant; D000072640:Interferon-Induced Helicase, IFIH1; D020382:Interleukin-18 / Q000097:blood; D007564:Japan; D017563:Lung Diseases, Interstitial / Q000097:blood* / Q000175:diagnosis* / Q000401:mortality; D008297:Male; D018396:Mucin-1 / Q000097:blood; D011237:Predictive Value of Tests; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D000888:Antibodies, Anti-Idiotypic; D015415:Biomarkers; D020382:Interleukin-18; C060192:MUC1 protein, human; D018396:Mucin-1; D005293:Ferritins; C452815:IFIH1 protein, human; D053487:DEAD-box RNA Helicases; D000072640:Interferon-Induced Helicase, IFIH1
keywords: KL-6; anti-melanoma differentiation-associated gene 5; ferritin; interleukin-18; interstitial lung disease; juvenile dermatomyositis