PANCREATIC INVOLVEMENT IN PATIENTS WITH SJOGRENS-SYNDROME AND PRIMARYBILIARY-CIRRHOSIS

Serum pancreatic enzyme activities, exocrine pancreatic function, and pancreatic ductal morphology were evaluated in patients with one or bo th of Sjogren's syndrome and primary biliary cirrhosis. Ten of 20 pati ents with Sjogren's syndrome (50%), 6 of 17 patients with primary bili ary cirrhosis (35%), and 4 of 11 patients with both diseases (36%) had an elevated level of at least one pancreatic enzyme, including elasta se-1, lipase, and trypsin. Diminished excretion of N-benzoyl-L-tyrosyl -para-aminobenzoic acid was observed in 3 of 17 patients with Sjogren' s syndrome (18%), 4 of 16 with primary biliary cirrhosis (25%), and no ne of 7 with both diseases. Endoscopic retrograde pancreatograms demon strated an abnormal pancreatic ductal configuration in 3 of ii patient s with Sjogren's syndrome (27%), 2 of 9 with primary biliary cirrhosis (22%), and 3 of 4 with both diseases (75%). Only minimal changes in b ranches of the pancreatic duct were observed in the pancreatogram. Fin ally, 9-30% of patients with Sjogren's syndrome and/or primary biliary cirrhosis had a mild and intermittent abdominal pain. These findings support the concept of a disease complex, ''autoimmune exocrinopathl,' ' in patients with Sjogren's syndrome, primary biliary cirrhosis, and chronic pancreatitis.