Primary sclerosing cholangitis associated with autoimmune pancreatitis.

We present 2 cases of primary sclerosing cholangitis complicated with autoimmune pancreatitis with narrowing of the pancreatic duct. In both cases, endoscopic retrograde cholangiography showed the characteristic findings of primary sclerosing cholangitis. In addition, positivity for autoantibody, hypergamma-globulinemia and narrowing of the pancreatic duct shown on endoscopic retrograde pancreatography were noticed in both. They were thu diagnosed as autoimmune pancreatitis. Whereas it is well known that primary sclerosing cholangitis is often complicated with chronic pancreatitis, it has rarely been studied whether the chronic pancreatitis is autoimmune pancreatitis or not. In English literature, possibly 7 equivalent cases have been reported so far. In those cases, the onset was in the post-prime of life, and the disease was frequently accompanied with Sjögren's syndrome, but not with ulcerative colitis; frankly the nature of the disease was slightly different from what has traditionally been thought as a typical primary sclerosing cholangitis. In primary sclerosing cholangitis complicated with autoimmune pancreatitis, the autoimmune mechanism seems to be much involved, and thus steroid therapy might be effective as in Case 2 in our series.