Table 1 Diagnostic criteria for IgG4 related lung disease

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