We reviewed the clinical features and
clinical course of patients with duct-narrowing
chronic pancreatitis who were negative for immunoserologic test results (n = 16) in comparison with the findings for serological test-positive patients (n = 20) in order to determine an adequate treatment for those who had typical morphology of
autoimmune pancreatitis in the absence of immunoserologic abnormality. No significant differences were found between the two groups of patients in clinical profiles including associated autoimmune-related
diseases, pancreatic histology, and response to
steroid therapy. Of the seronegative patients, eight who showed an improvement in narrowing of the main pancreatic duct with
steroid therapy and three who did no show an improvement or who relapsed after surgical resection without this
therapy had
stenosis of the common bile duct with increased levels of serum hepatobiliary
enzymes, except for two patients with affected sites limited to the body or tail of the gland. For the remaining five patients, who showed an improvement in pancreatic duct changes or long-term remission after surgery without
steroid administration, normal biochemistry test results for liver functions were obtained, with no abnormal cholangiographic findings in the three patients examined. Duct-narrowing
chronic pancreatitis without immunoserologic abnormality overlaps in clinical features with that fulfilling the immunoserologic criteria for a diagnosis of
autoimmune pancreatitis. In particular, the disease with bile duct involvement should be treated clinically as
autoimmune pancreatitis, for which
steroid therapy is recommended, even if an autoimmune mechanism is not demonstrated serologically.