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Immunoglobulin G4-related lung disease: clinicoradiological and pathological features.
MedLine Citation:
PMID:  23145930     Owner:  NLM     Status:  Publisher    
SUMMARY AT A GLANCE: The clinicoradiological and pathological features of IgG4-related lung disease were assessed. Clinically, this disease is silent, although radiological and pathological examinations showed that it involves lymphatic routes, with spread observed on imaging and infiltration on pathological examination. Clinicoradiological and pathological correlation is necessary for the diagnosis of this disease. ABSTRACT: Background and 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, we have assessed the clinical, radiological and pathological features of patients with this disease. 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:  Our findings suggest that the intrathoracic manifestations of IgG4-related lung disease develop through lymphatic routes of the lungs and show various clinical characteristics. Since some lymphoproliferative disorders show similar findings, the correlation of clinicoradiological and pathological characteristics is crucial for the diagnosis of IgG4-related lung disease. © 2012 The Authors. Respirology © 2012 Asian Pacific Society of Respirology.
Shoko Matsui; Akira Hebisawa; Fumikazu Sakai; Horoshi Yamamoto; Yasuhiro Terasaki; Yasuyuki Kurihara; Yuko Waseda; Tetsuji Kawamura; Tomoko Miyashita; Hiromasa Inoue; Norihiko Hata; Hiroaki Masubuchi; Keishi Sugino; Jun Kishi; Hideo Kobayashi; Yutaka Usui; Yoshitoshi Komazaki; Yoshinori Kawabata; Takashi Ogura
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-13
Journal Detail:
Title:  Respirology (Carlton, Vic.)     Volume:  -     ISSN:  1440-1843     ISO Abbreviation:  Respirology     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9616368     Medline TA:  Respirology     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2012 The Authors. Respirology © 2012 Asian Pacific Society of Respirology.
Health Administration Center, University of Toyama, Toyama, Japan Division of Clinical Pathology, NHO Tokyo National Hospital, Tokyo, Japan Department of Radiology, Saitama International Medical Center, Saitama Medical University, Saitama, Japan First Department of Internal Medicine, Shinshu University School of Medicine, Nagano, Japan Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan Department of Radiology, St. Marianna University School of Medicine, Kanagawa, Japan Department of Respiratory Medicine, Kanazawa University School of Medicine, Ishikawa, Japan Department of Respiratory Medicine, NHO Himeji Medical Center, Hyogo, Japan Department of Internal Medicine, Osaka City General Hospital, Osaka, Japan Department of Pulmonary Medicine, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan Department of Internal Medicine, Kawaguchi Municipal Medical Center, Saitama, Japan Department of Medicine and Biological Science, Gunma University Graduate School of Medicine, Gunma, Japan Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Department of Respiratory Medicine and Rheumatology, The University of Tokushima Graduate School,Tokushima, Japan Division of Respiratory Medicine, National Defense Medical College, Saitama, Japan Department of Respiratory Disease, Saitama Medical University, Saitama, Japan Department of Integrated Pulmonology, Tokyo Medical and Dental University, Tokyo, Japan, Division of Diagnostic Pathology, Saitama Prefectural Cardiovascular and Respiratory Center, Saitama, Japan Division of Respiratory Medicine, Kanagawa Prefectural Cardiovascular and Respiratory Center, Kanagawa, Japan.
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