Clinical Profile of Autoimmune Pancreatitis and Its Histological Subtypes: An International Multicenter Survey : Pancreas

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Clinical Profile of Autoimmune Pancreatitis and Its Histological Subtypes

An International Multicenter Survey

Kamisawa, Terumi MD, PhD*; Chari, Suresh T. MD, PhD; Giday, Samuel A. MD, PhD; Kim, Myung-Hwan MD, PhD§; Chung, Jae Bock MD, PhD; Lee, Kyu Taek MD, PhD; Werner, Jens MD#; Bergmann, Frank MD**; Lerch, Markus M. MD††; Mayerle, Julia MD††; Pickartz, Tilman MD††; Lohr, Matthias MD‡‡; Schneider, Alexander MD§§; Frulloni, Luca MD, PhD∥∥; Webster, George J. M. MD, PhD¶¶; Reddy, D. Nageshwar MD, PhD##; Liao, Wei-Chih MD, PhD***; Wang, Hsiu-Po MD, PhD***; Okazaki, Kazuichi MD, PhD†††; Shimosegawa, Tooru MD, PhD‡‡‡; Kloeppel, Guenter MD§§§; Go, Vay Liang W. MD∥∥∥

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Pancreas 40(6):p 809-814, August 2011. | DOI: 10.1097/MPA.0b013e3182258a15

Abstract

Objective: 

The objective of this study was to clarify the clinical and pathophysiological characteristics of autoimmune pancreatitis (AIP) and its subtypes (lymphoplasmacytic sclerosing pancreatitis [LPSP] and idiopathic duct-centric pancreatitis [IDCP]) seen around the world.

Methods: 

An international multicenter survey of AIP was conducted in 15 institutes from 8 countries. We compared clinical and pathologic profiles of AIP (n = 731) and the clinical profiles of LPSP (n = 204) and IDCP (n = 64) patients.

Results: 

Patients with LPSP were approximately 16 years older than IDCP patients. Obstructive jaundice was a more frequent presentation in LPSP versus IDCP (75% vs 47%, P < 0.001), whereas abdominal pain (41% vs 68%, P < 0.001) and acute pancreatitis (5% vs 34%, P < 0.001) were more frequent in IDCP patients. Patients with LPSP were more likely to have diffuse swelling of the pancreas (40% vs 25%, P = 0.037) and elevated serum IgG4 levels (63% vs 23%, P < 0.001) but less likely to be associated with ulcerative colitis (1% vs 16%, P < 0.001). Clinical profiles of non-histologically confirmed AIP from Asia, the United States, and United Kingdom corresponded with that of LPSP, whereas those from Italy and Germany suggested a mixture of LPSP and IDCP.

Conclusions: 

Autoimmune pancreatitis is seen all around the world, with regional differences in the pathologic and clinical features. Lymphoplasmacytic sclerosing pancreatitis and IDCP have distinct clinical profiles.

© 2011 Lippincott Williams & Wilkins, Inc.

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