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Acute pancreatitis as the first presentation of Wegener's granulomatosis

Acute pancreatitis as the first presentation of Wegener's granulomatosis
Acute pancreatitis as the first presentation of Wegener's granulomatosis
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.

pancreatitis, acute necrotizing, wegener granulomatosis, vasculitis
1590-8577
300-304
Abu-Hilal, Mohammed
384e1c60-8519-4eed-8e92-91775aad4c47
McPhail, Mark J.W.
3a321e07-0e55-495f-b708-9da8beab18ad
Zeidan, Bashar
acd18415-22ee-43b8-a102-a36ea22dd0af
Bryant, Tim
0ae39e3b-3a9f-4385-865d-a764f64baebd
Bateman, Adrian
35f5fef8-6358-42ff-b3c1-2ff226b4dc43
Johnson, Colin D.
e50aa9cd-8c61-4fe3-a0b3-f51cc3a6c74a
Abu-Hilal, Mohammed
384e1c60-8519-4eed-8e92-91775aad4c47
McPhail, Mark J.W.
3a321e07-0e55-495f-b708-9da8beab18ad
Zeidan, Bashar
acd18415-22ee-43b8-a102-a36ea22dd0af
Bryant, Tim
0ae39e3b-3a9f-4385-865d-a764f64baebd
Bateman, Adrian
35f5fef8-6358-42ff-b3c1-2ff226b4dc43
Johnson, Colin D.
e50aa9cd-8c61-4fe3-a0b3-f51cc3a6c74a

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)

Record type: Article

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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More information

Published date: 2008
Keywords: pancreatitis, acute necrotizing, wegener granulomatosis, vasculitis

Identifiers

Local EPrints ID: 172245
URI: http://eprints.soton.ac.uk/id/eprint/172245
ISSN: 1590-8577
PURE UUID: efae153c-dccc-4fe8-b0af-fcfb55c8a0fa

Catalogue record

Date deposited: 25 Jan 2011 10:12
Last modified: 08 Jan 2022 08:39

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Contributors

Author: Mohammed Abu-Hilal
Author: Mark J.W. McPhail
Author: Bashar Zeidan
Author: Tim Bryant
Author: Adrian Bateman

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