A number of lupus patients develop episodes of acute "idiopathic" pancreatitis, unrelated to the known causes of mechanical obstruction of the pancreatic duct or toxic-metabolic etiologies. This lupus-associated pancreatitis is rare. The estimated annual incidence was 0.4-1.1/1000 lupus patients. A literature search found detailed descriptions of this condition in 77 lupus patients. Their median age was 27, and 88% were females. Abdominal pain was the most frequent pancreatitis-related symptom (88%). In 97% the diagnosis of pancreatitis was based on laboratory evidence of elevated serum amylase or lipase. Most cases were unrelated to treatment with steroids or azathioprine. Most of the patients (84%) had active lupus at the time of pancreatitis. Mortality rate was 27%, higher than in non-SLE associated pancreatitis. Active lupus and several biochemical abnormalities, but not treatment with steroids or azathioprine, were significantly associated with increased mortality. Treatment with steroids lowered the mortality by 67% compared to non-treated patients.