Acute intermittent porphyria with pancreatitis and pancreatic fistula

Abstract

Objective To report a case of acute intermittent porphyria and acute, relapsing pancreatitis with pancreatic fistula. Design Single case report and review of the literature. Setting Medical department of a community hospital. Patient A 39-year-old woman with recurrent attacks of abdominal pain. On the first occasion, she was diagnosed as having acute pancreatitis. During the second episode of abdominal pain, a diagnosis of acute intermittent porphyria could be established and a pancreatico-peritoneal fistula was documented by endoscopic retrograde cholangiopancreatography. Outcome The patient was discharged from hospital without symptoms. Conclusion To our knowledge, this is the first reported case of acute intermittent porphyria with acute pancreatitis and pancreatic fistula. This report adds to the scarce data on the association of porphyria and pancreatitis. Acute intermittent porphyria may be obscured by the presence of acute pancreatitis, especially when there is a delay between the onset of abdominal pain and laboratory evaluation, since an increase in pancreatic enzyme levels occurs late in the course of a porphyric attack.