Primary antiphospholipid syndrome and necrotizing pancreatitis: a diagnostic challenge.

The objective of this study was to report an unusual case of primary antiphospholipid syndrome (APS)-associated severe necrotizing pancreatitis. Since the APS was first recognized in the 1980s, a number of manifestations of the disorder have been described. We report primary APS presenting as severe necrotizing pancreatitis. This is the first such case to date that fulfills the revised Sapporo classification criteria. A 38-year-old previously healthy woman presented with new-onset hypertensive emergency and acute kidney injury. She subsequently developed severe epigastric pain attributable to necrotizing pancreatitis and extensive splenic infarcts. Biopsies of both the pancreas and kidney revealed thrombotic microangiopathy. Her lupus anticoagulant was positive on both weeks 1 and 12 of her disease course. A diagnosis of primary APS was made. Despite 6 months of aggressive care, she died of sepsis.