Elevation of serum IgG4 in Western patients with autoimmune sclerosing pancreatocholangitis: a word of caution.
BACKGROUND
Autoimmune pancreatocholangitis is characterized by sclerosing inflammation of the biliary tree or pancreatic duct and can mimic pancreaticobiliary malignancy. Serum immunoglobin (Ig) G4 values seem to be helpful in distinguishing autoimmune pancreatocholangitis from pancreatic malignancy in the Japanese population; however, its significance in the Western population has not been well studied.


METHODS
We report a retrospective analysis of 7 consecutive patients with autoimmune pancreatocholangitis and compare them to 23 patients with pancreatic malignancy. Clinical presentation, diagnostic tests, and preoperative IgG4 levels were reviewed in all patients. Presence of autoimmune pancreatocholangitis or pancreatic malignancy was determined by pathologic analysis in all patients and reviewed by a single pathologist.


RESULTS
In all patients, autoimmune pancreatocholangitis manifested in a similar fashion to pancreatic malignancy. Median IgG4 levels were far lower in pancreatic cancer patients with localized, resectable disease (24 mg/dL), locally advanced disease (24 mg/dL), and metastatic disease (28 mg/dL) as compared with patients with autoimmune pancreatocholangitis (142 mg/dL, P < .05). Only one patient with pancreatic cancer had an IgG4 level that was >100 mg/dL. In contrast, all patients with autoimmune pancreatitis or cholangitis had levels >100 mg/dL. However, in five of these seven patients, IgG4 levels were below the upper limits of normal.


CONCLUSIONS
Autoimmune pancreatocholangitis mimics pancreatobiliary malignancy. Serum IgG4 values seem to be helpful in distinguishing autoimmune pancreatocholangitis from malignancy in the Western population. However, absolute values seem to be lower in the United States compared with Japan. The upper limit of normal as reported in laboratories in the United States may not be useful in identifying abnormally high IgG4 values. A new upper limit of normal may need to be defined because IgG subclass determinations are being used more frequently in Western patients with biliary obstruction.
Hochwald
Steven N
SN
Division of Surgical Oncology, Department of Surgery, University of Florida College of Medicine, 1600 SW Archer Road, 100286, Gainesville, Florida, USA. steven.hochwald@surgery.ufl.edu
Hemming
Alan W
AW
Draganov
Peter
P
Vogel
Stephen B
SB
Dixon
Lisa R
LR
Grobmyer
Stephen R
SR
D000293:Adolescent; D000368:Aged; D001327:Autoimmune Diseases / Q000097:blood* / Q000601:surgery; D015209:Cholangitis, Sclerosing / Q000097:blood* / Q000601:surgery; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000097:blood* / Q000601:surgery; D012016:Reference Values; D012189:Retrospective Studies; D014481:United States
D007074:Immunoglobulin G