title: Immunoglobulin G4-related lung disease: clinicoradiological and pathological features.
abstract: OBJECTIVE
  Immunoglobulin G4 (IgG4)-related disease is a multi-organ disorder that can include the lungs. IgG4-related lung disease can present in various forms; the clinical, radiological and pathological features of patients with this disease have been assessed.
  
  
  METHODS
  Forty-eight patients suspected of having IgG4-related lung disease, with a high serum concentration of IgG4 and abundant IgG4-positive plasma cell infiltration into the intrathoracic organs, were retrospectively evaluated. Their clinical features, chest imaging findings and pathological findings were examined, with final diagnoses made by an open panel conference.
  
  
  RESULTS
  Of the 48 patients, 18 with extrathoracic manifestations were diagnosed as having IgG4-related lung disease. Most of these patients were middle-aged to elderly men. IgG4-related lung disease was characterized by high serum concentrations of IgG and IgG4, normal white blood cell count and serum C-reactive protein concentration and a good response to corticosteroids. Common radiological findings included mediastinal lymphadenopathy and thickening of the perilymphatic interstitium, with or without subpleural and/or peribronchovascular consolidation. Pathological examination showed massive lymphoplasmacytic infiltration with fibrosis in and around the lymphatic routes, with distribution well correlated with radiological manifestations.
  
  
  CONCLUSIONS
  The findings suggest that the intrathoracic manifestations of IgG4-related lung disease develop through lymphatic routes of the lungs and show various clinical characteristics. Because some lymphoproliferative disorders show similar findings, the correlation of clinicoradiological and pathological characteristics is crucial for the diagnosis of IgG4-related lung disease.
authors.affiliation: Health Administration Center, University of Toyama, Toyama, Japan. smatsui@med.u-toyama.ac.jp
authors.forename: Shoko
authors.initials: S
authors.lastname: Matsui
authors.forename: Akira
authors.initials: A
authors.lastname: Hebisawa
authors.forename: Fumikazu
authors.initials: F
authors.lastname: Sakai
authors.forename: Horoshi
authors.initials: H
authors.lastname: Yamamoto
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Terasaki
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Kurihara
authors.forename: Yuko
authors.initials: Y
authors.lastname: Waseda
authors.forename: Tetsuji
authors.initials: T
authors.lastname: Kawamura
authors.forename: Tomoko
authors.initials: T
authors.lastname: Miyashita
authors.forename: Hiromasa
authors.initials: H
authors.lastname: Inoue
authors.forename: Norihiko
authors.initials: N
authors.lastname: Hata
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Masubuchi
authors.forename: Keishi
authors.initials: K
authors.lastname: Sugino
authors.forename: Jun
authors.initials: J
authors.lastname: Kishi
authors.forename: Hideo
authors.initials: H
authors.lastname: Kobayashi
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Usui
authors.forename: Yoshitoshi
authors.initials: Y
authors.lastname: Komazaki
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Kawabata
authors.forename: Takashi
authors.initials: T
authors.lastname: Ogura
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D001999:Bronchoscopy; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D008168:Lung / Q000000981:diagnostic imaging* / Q000473:pathology*; D008171:Lung Diseases / Q000175:diagnosis / Q000276:immunology* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000175:diagnosis / Q000276:immunology* / Q000378:metabolism; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D013902:Radiography, Thoracic / Q000379:methods*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
chemical_list: D007074:Immunoglobulin G