The patients with
autoimmune pancreatitis usually present with
jaundice and a pancreatic head mass, presumed to have
pancreatic cancer, and they often undergo pancreatic resection. Elevated serum
IgG4 levels (>135 mg/dL) help to distinguish
autoimmune pancreatitis from
pancreatic cancer. However, when the biopsy from a pancreatic mass shows dense chronic
inflammation and
fibrosis and the serum
IgG4 level is not available, it presents a diagnostic dilemma whether it represents
autoimmune pancreatitis or peritumoral
pancreatitis. We performed
IgG4 immunohistochemistry on 25 cases of
autoimmune pancreatitis-lymphoplasmacytic sclerosing
pancreatitis, 7 cases of
autoimmune pancreatitis with granulocytic epithelial lesions, 8 cases of nonspecific
pancreatitis, 15 cases of
pancreatitis associated with pancreatic ductal
adenocarcinoma, and 5 biopsies of pancreatic
adenocarcinoma with variable
inflammation. The distribution of IgG4-positive cells was noted in each case. Eighty-four percent (21/25) of
autoimmune pancreatitis-LPSP cases showed diffuse and dense staining for
IgG4, with more than 50 positive plasma cells per high-power field (range, 50-150 cells/hpf) in the highest density area. Most (5/7) cases of
autoimmune pancreatitis-granulocytic epithelial lesions were negative for
IgG4. Thirty-nine percent of nonspecific
pancreatitis and peritumoral
pancreatitis cases stained positive for
IgG4, but the distribution was focal and none of the cases showed more than 50 IgG4-positive cells/hpf in the highest density area of
IgG4 staining. IgG4-positive cells in peritumoral
pancreatitis and nonspecific
pancreatitis cases were closely associated with malignant glands and areas of acute
inflammation in some cases. Using a cutoff of 50 IgG4-positive cells/hpf, the sensitivity of
IgG4 staining for classical
autoimmune pancreatitis-LPSP versus other types of
pancreatitis was 84%, the specificity was 100%, and the P value was significant (<.0001). Hence, we conclude that diffuse and dense staining (>50 positive cells/hpf) for
IgG4 is specifically seen in
autoimmune pancreatitis-LPSP, and
IgG4 staining along with the histologic features and serum
IgG4 levels may be very helpful in diagnosing
autoimmune pancreatitis.