Table 2.

Spectrum of diagnoses associated with high serum IgG4 Levels (n = 158).

Diagnosis	No. of patients (%)
IgG4-related disease	29 (18.4)
Definite	10
Probable	0
Possible	19
Respiratory diseases	32 (20.3)
Bronchiectasis	11
Chronic rhinosinusitis	7
Asthma	4
Idiopathic pulmonary fibrosis	3
Sarcoidosis	2
Other respiratory diseases*	5
Biliary tract diseases	26 (16.5)
Primary sclerosing cholangitis	17
Cholangiocarcinoma	6
Biliary stricture or stone	3
Pancreatic diseases	19 (12.0)
Pancreatitis, not IgG4 related	10
Pancreatic cancer	6
Other pancreatic diseases†	3
Cirrhosis and other liver diseases	9 (5.7)
Vasculitis	9 (5.7)
Granulomatosis with polyangiitis (Wegener's)	5
The Churg-Strauss syndrome	3
Polyarteritis nodosa	1
Atopic dermatitis	1 (0.6)
Miscellaneous diseases‡	4 (2.5)
No specific diagnosis	29 (18.4)

*Other respiratory diseases (5 patients) included chronic pleuritis, emphysema, fibrosing mediastinitis, hypersensitivity pneumonitis, and recurrent pneumonias, respectively.

^†Other pancreatic diseases (3 patients) included intraductal papillary mucinous neoplasm, pancreatic cyst, and pancreatic insufficiency, respectively.

^‡Miscellaneous diseases (4 patients) included lactose intolerance, neurofibromatosis, polymyositis, and psoriasis, respectively.