1. Abnormal shadow on chest CT |
・Hilar/mediastinal lymphadenopaty |
・Thickening of bronchial wall, bronchovascular bundle, interlobular septal wall |
・Nodular shadow, infiltrative shadow, pleural thickening/effusion |
2. Elevated serum IgG4 concentration (> 135 mg/dL) |
3. Pathological findings satisfing the following two items or more: a: >three items, b: two items |
1) Dense lymphoplasmacytic cell infiltration into respiratory organ tissues |
2) IgG4+/IgG + cell ratio > 40% and/or > 10 IgG4 + cells/high power field |
3) Obliterative phlebitis or obliterative arteritis |
4) Characteristics fibrosis, typically storiform pattern |
4. Presence of lesions in the extrathoracic organs satisfying the diagnostic criteria of IgG4-related disease |
<reference finding > hypocomplemenemia |