title: Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy.
abstract: Anti-glycyl-tRNA synthetase (anti-EJ) antibody is occasionally positive in patients with interstitial lung disease (ILD). We aimed to define the clinical, radiological and pathological features of patients with anti-EJ antibody-positive ILD (EJ-ILD).
  
  
  
  We retrospectively analyzed the medical records of 12 consecutive patients with EJ-ILD who underwent surgical lung biopsy.
  
  
  
  The median follow-up time was 74 months (range, 17-115 months). The median age was 62 years (range, 47-75 years). Seven of 12 patients were female. Eight patients presented with acute onset. Six patients eventually developed polymyositis/dermatomyositis. On high-resolution computed tomography, consolidation and volume loss were predominantly observed in the middle or lower lung zone. Nine patients presented pathologically nonspecific interstitial pneumonia with organizing pneumonia, alveolar epithelial injury and prominent interstitial cellular infiltrations whereas the other three patients were diagnosed with unclassifiable interstitial pneumonia. Although all patients but one improved with the initial immunosuppressive therapy, five patients relapsed. When ILD relapsed, four of the five patients were treated with corticosteroid monotherapy. Four of the six patients without relapse have been continuously treated with combination therapy of corticosteroid and immunosuppressant.
  
  
  
  Patients with EJ-ILD often had acute onset of ILD with lower lung-predominant shadows and pathologically nonspecific interstitial pneumonia or unclassifiable interstitial pneumonia with acute inflammatory findings. Although the disease responded well to the initial treatment, relapse was frequent. Because of the diversity of the clinical courses, combination therapy of corticosteroid and immunosuppressant should be on the list of options to prevent relapse of EJ-ILD.
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan.|Present Address: Department of Respiratory Medicine, Ise Red Cross Hospital, 1-471-2 Funae, Ise, 516-8512, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Sasano
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan.
authors.forename: Eri
authors.initials: E
authors.lastname: Hagiwara
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan.
authors.forename: Hideya
authors.initials: H
authors.lastname: Kitamura
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan.|Present Address: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-Ku, Hamamatsu, 431-3192, Japan.
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Enomoto
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan.|Present Address: Department of Respirology, Kurume University School of Medicine, 67 Asahi-Chō, Kurume, 830-0011, Japan.
authors.forename: Norikazu
authors.initials: N
authors.lastname: Matsuo
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan.
authors.forename: Tomohisa
authors.initials: T
authors.lastname: Baba
authors.affiliation: Department of Radiology, Yokohama Rosai Hospital for Labor Welfare Corporation, 3211 Kozukue-Chō, Kōhoku-Ku, Yokohama, 222-0036, Japan.
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Iso
authors.affiliation: Department of Pathology, Yokohama City University Graduate School of Medicine, 3-9, Fukuura, Kanazawa-Ku, Yokohama, 236-0004, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Okudela
authors.affiliation: Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan.
authors.forename: Tae
authors.initials: T
authors.lastname: Iwasawa
authors.affiliation: Department of Rheumatology, Tokai University School of Medicine, 143 Shimokasuya, Isehara, 259-1193, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Sato
authors.affiliation: Department of Rheumatology, Tokai University School of Medicine, 143 Shimokasuya, Isehara, 259-1193, Japan.
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Suzuki
authors.affiliation: Department of Pathology, Japan Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-Ku, Tokyo, 150-8935, Japan.
authors.forename: Tamiko
authors.initials: T
authors.lastname: Takemura
authors.affiliation: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan. takashiogurajunko@yahoo.co.jp.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ogura
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D001706:Biopsy; D003882:Dermatomyositis / Q000209:etiology; D005260:Female; D006032:Glycine-tRNA Ligase / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D006032:Glycine-tRNA Ligase
keywords: Anti-synthetase syndrome; Dermatomyositis; Idiopathic interstitial pneumonia; Interstitial pneumonia with autoimmune featured (IPAF); Polymyositis