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Immunoglobulin G4 ‐related lung disease: Clinicoradiological and pathological features
Author(s) -
Matsui Shoko,
Hebisawa Akira,
Sakai Fumikazu,
Yamamoto Horoshi,
Terasaki Yasuhiro,
Kurihara Yasuyuki,
Waseda Yuko,
Kawamura Tetsuji,
Miyashita Tomoko,
Inoue Hiromasa,
Hata Norihiko,
Masubuchi Hiroaki,
Sugino Keishi,
Kishi Jun,
Kobayashi Hideo,
Usui Yutaka,
Komazaki Yoshitoshi,
Kawabata Yoshinori,
Ogura Takashi
Publication year - 2013
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.12016
Subject(s) - medicine , pathological , igg4 related disease , pathology , lung , fibrosis , lymphatic system , disease , mediastinal lymphadenopathy , biopsy
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; 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.