Pancreatic pseudotumor with sclerosing pancreato-cholangitis: is this a systemic disease?

OBJECTIVE AND METHOD: Primary sclerosing cholangitis (PSC) is a disease that predominantly affects the biliary tree, although the pancreas may also be affected. A review of the presenting features of all patients given a diagnosis of PSC at a single center was conducted. The aim was to clarify the presentation of patients with pseudotumor of the pancreas in this patient population. RESULTS: Seventy-two patients were diagnosed with PSC either by ERCP (63/72 = 88%) or by liver biopsy (9/72 = 12%). The diagnosis of PSC was made following referral for abnormal liver tests (67%), jaundice (17%), and acute cholangitis (5%). Inflammatory bowel disease (IBD) (60%), non-insulin-dependent diabetes mellitus (NIDDM) (13%), thyroid disease (8%), and pancreatic disease (7%) were the major coexistent extrahepatic diseases. Three patients, all with marked weight loss, who presented with jaundice, abdominal pain, and/or diarrhea were found to have a pancreatic mass at first presentation. Clinical and radiological findings suggested pancreatic malignancy, and only later was advanced sclerosing cholangitis identified. The biopsy of the pancreas in two of these three patients revealed chronic pancreatitis. The long-term follow-up and good clinical response to medical therapy confirmed lack of pancreatic malignancy. These three patients all had other evidence of systemic involvement: submandibular gland fibrosis and urethral stricture in one, fibromuscular dysplasia of the renal artery in another, and retroperitoneal fibrosis in the third. None had IBD. CONCLUSION: Pancreatic pseudotumor with sclerosing pancreato-cholangitis may be a manifestation of a systemic disease characterized by nonmalignant strictures and multifocal fibroinflammatory processes, unlike classical PSC.