Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey
Authors
Terumi Kamisawa ; Suresh T. Chari ; Samuel A. Giday ; Myung-Hwan Kim ; Jae Bock Chung ; Kyu Taek Lee ; Jens Werner ; Frank Bergmann ; Markus M. Lerch ; Julia Mayerle ; Tilman Pickartz ; Matthias Lohr ; Alexander Schneider ; Luca Frulloni ; George J. M. Webster ; D. Nageshwar Reddy ; Wei-Chih Liao ; Hsiu-Po Wang ; Kazuichi Okazaki ; Tooru Shimosegawa ; Guenter Kloeppel ; Vay Liang W. Go
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.