Acute pancreatitis as the first presentation of Wegener's granulomatosis

Abu-Hilal, Mohammed, McPhail, Mark J.W., Zeidan, Bashar, Bryant, Tim, Bateman, Adrian and Johnson, Colin D. (2008) Acute pancreatitis as the first presentation of Wegener's granulomatosis. JOP: Journal of the Pancreas, 9 (3), 300-304. (PMID:18469442)

Abstract

Context Wegener’s granulomatosis is a systemic vasculitis with prominent involvement of the respiratory tract and kidney. An association between acute pancreatitis and Wegener's granulomatosis is rarely reported and is even rarer as the first presentation. This can result in diagnostic difficulty and may allow severe pancreatitis to develop with potentially poor outcome.

Case report We report a rare case with fatal outcome of vasculitis consistent with Wegener’s granulomatosis presenting as acute pancreatitis in a 20-year-old female. The patient was admitted with worsening abdominal pain associated with nausea and loss of appetite. Accepted causes of acute pancreatitis were excluded and granulomatous vasculitis of the pancreas was confirmed from immunological profile, computed tomography and histology. As the disease progressed the patient experienced cutaneous, pulmonary, renal and severe gut involvement. Thirteen months from diagnosis the patient died of multi-organ failure despite appropriate surgical and immunosuppressive therapy.

Conclusion Vasculitic disease of the pancreas is rare but should be considered when other causes have been appropriately ruled out. Careful radiological, immunological and histological diagnosis is necessary and early immunosuppressant therapy in conjunction with advice from immunologists is essential to avoid the poor outcome reported in this and other case reports.

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