PT  - JOURNAL ARTICLE
AU  - Yuko Waseda
AU  - Shoko Matsui
AU  - Hiroshi Yamamoto
AU  - Takashi Ogura
AU  - Akira Hebisawa
AU  - Fumikazu Sakai
AU  - Yasuhiro Terasaki
AU  - Yasuyuki Kurihara
AU  - Kingo Chida
AU  - Yohei Yatagai
AU  - Fumiya Fukushima
AU  - Mutsuo Kuba
AU  - Koichi Miyashita
AU  - Mikio Toyoshima
AU  - Masayuki Ishida
AU  - Hiroki Hayashi
AU  - Yutaka Tsuchiya
AU  - Keishi Ohishi
AU  - Yoshiaki Morio
AU  - Kenji Konishi
AU  - Masaki Fujimura
TI  - Lung-limited IgG4-related disease: A new form of IgG4-related disease?
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - P1775
VI  - 40
IP  - Suppl 56
4099  - http://erj.ersjournals.com/content/40/Suppl_56/P1775.short
4100  - http://erj.ersjournals.com/content/40/Suppl_56/P1775.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - Background: 'Immunoglobulin G4 (IgG4)-related disease (IgG4-RD)' comprises multi-organ diseases including pulmonary disorders. Typical patients show extrathoracic lesions. We compared cases with or without extrathoracic lesions. Method: Tokyo Diffuse Lung Diseases Study Group retrospectively examined data from 44 patients suspected of IgG4-RD. Diagnostic criteria included high serum IgG4 level (>135mg/dL). Lung biopsy specimens showed massive IgG4+ plasma cell infiltrations (IgG4+/IgG+ >40% and >10/high power fields). Computed tomography and pathological findings were evaluated by diagnostic radiologists and pathologists independently. Final diagnoses were made by open panel conference. Result: Of 44 patients, 20 had extrathoracic lesions and 24 had intrathoracic lesions alone. We classified 20 extrathoracic lesion cases as IgG4-related RD (A group) and 15/24 cases without extrathoracic lesions as suspected disease entity such as non-specific interstitial pneumonia (B group). In A, radiological findings included hilomediastinal lymphadenopathy, bronchial wall and bronchovascular (BV) bundles thickening, interlobular septal thickening and/or periBV consolidation. Pathological findings showed abundant lymphoplasmacytic inflammation in interlobular septa, periBV interstitium, bronchus and pleura. Phlebitis, angiitis, granulation tissue, and/or fibrosis were also observed. The remaining 9 (C group) showed similar pulmonary involvement as A excluding extrathoracic lesions. Conclusions: There is C group with similar radiological and pathological features as A excluding extrathoracic lesions, and it might be “lung-limited IgG4-RD”. Further discussion is necessary for diagnostic consensus of lung-limited disease.