Diagnosis of Autoimmune Pancreatitis: The Mayo Clinic Experience

Suresh T Chari, Thomas Christopher Smyrk, Michael J. Levy, Mark Topazian, Naoki Takahashi, Lizhi Zhang, Jonathan E. Clain, Randall K. Pearson, Bret Thomas Petersen, Santhi Swaroop Vege, Michael B. Farnell

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Abstract

Background & Aims: The Japan Pancreas Society criteria for diagnosis of autoimmune pancreatitis (AIP) mandate presence of characteristic imaging (diffuse pancreatic enlargement with diffusely irregular, narrow pancreatic duct). AIP has unique histologic features associated with infiltration of tissues of affected organs with abundant IgG4-positive cells. We propose expanded diagnostic criteria for AIP with a cohort of histologically confirmed AIP. Methods: We reviewed the pancreatic imaging findings on computed tomography scans, serum IgG4 levels, other organ involvement, and response to steroids in 29 consecutive patients who met histologic criteria for AIP. Results: Computed tomography scans (n = 22) showed diffuse pancreatic enlargement in 6 (27%) patients; the rest had focal enlargement, distinct mass, normal pancreas, or focal acute pancreatitis. Serum IgG4 level was elevated in 15 of 21 (71%) patients, and other organ involvement (eg, intrahepatic biliary strictures) was noted in 11 of 29 (38%) patients. All 17 patients treated with steroids exhibited resolution/marked improvement of pancreatic/extrapancreatic manifestation. On the basis of this experience we propose that diagnosis of AIP can be made in patients with ≥1 of these criteria: (1) diagnostic histology, (2) characteristic imaging on computed tomography and pancreatography with elevated serum IgG4 level, or (3) response to steroid therapy of pancreatic/extrapancreatic manifestations of AIP. Twenty additional patients met expanded diagnostic criteria for AIP, and their demographic and clinical profile was similar to that of the 29 patients meeting histologic criteria. Conclusions: AIP defined by histological criteria shows a wide spectrum of radiologic features, with characteristic imaging seen only in a minority. Incorporation of additional features into current diagnostic criteria can identify the full spectrum of clinical presentations of AIP.

Original languageEnglish (US)
Pages (from-to)1010-1016
Number of pages7
JournalClinical Gastroenterology and Hepatology
Volume4
Issue number8
DOIs
StatePublished - Aug 2006

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Pancreatitis
Immunoglobulin G
Steroids
Tomography
Pancreas
Serum
Pancreatic Ducts
Histology
Japan
Pathologic Constriction
Demography

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Diagnosis of Autoimmune Pancreatitis : The Mayo Clinic Experience. / Chari, Suresh T; Smyrk, Thomas Christopher; Levy, Michael J.; Topazian, Mark; Takahashi, Naoki; Zhang, Lizhi; Clain, Jonathan E.; Pearson, Randall K.; Petersen, Bret Thomas; Vege, Santhi Swaroop; Farnell, Michael B.

In: Clinical Gastroenterology and Hepatology, Vol. 4, No. 8, 08.2006, p. 1010-1016.

Research output: Contribution to journalArticle

Chari, Suresh T ; Smyrk, Thomas Christopher ; Levy, Michael J. ; Topazian, Mark ; Takahashi, Naoki ; Zhang, Lizhi ; Clain, Jonathan E. ; Pearson, Randall K. ; Petersen, Bret Thomas ; Vege, Santhi Swaroop ; Farnell, Michael B. / Diagnosis of Autoimmune Pancreatitis : The Mayo Clinic Experience. In: Clinical Gastroenterology and Hepatology. 2006 ; Vol. 4, No. 8. pp. 1010-1016.
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abstract = "Background & Aims: The Japan Pancreas Society criteria for diagnosis of autoimmune pancreatitis (AIP) mandate presence of characteristic imaging (diffuse pancreatic enlargement with diffusely irregular, narrow pancreatic duct). AIP has unique histologic features associated with infiltration of tissues of affected organs with abundant IgG4-positive cells. We propose expanded diagnostic criteria for AIP with a cohort of histologically confirmed AIP. Methods: We reviewed the pancreatic imaging findings on computed tomography scans, serum IgG4 levels, other organ involvement, and response to steroids in 29 consecutive patients who met histologic criteria for AIP. Results: Computed tomography scans (n = 22) showed diffuse pancreatic enlargement in 6 (27{\%}) patients; the rest had focal enlargement, distinct mass, normal pancreas, or focal acute pancreatitis. Serum IgG4 level was elevated in 15 of 21 (71{\%}) patients, and other organ involvement (eg, intrahepatic biliary strictures) was noted in 11 of 29 (38{\%}) patients. All 17 patients treated with steroids exhibited resolution/marked improvement of pancreatic/extrapancreatic manifestation. On the basis of this experience we propose that diagnosis of AIP can be made in patients with ≥1 of these criteria: (1) diagnostic histology, (2) characteristic imaging on computed tomography and pancreatography with elevated serum IgG4 level, or (3) response to steroid therapy of pancreatic/extrapancreatic manifestations of AIP. Twenty additional patients met expanded diagnostic criteria for AIP, and their demographic and clinical profile was similar to that of the 29 patients meeting histologic criteria. Conclusions: AIP defined by histological criteria shows a wide spectrum of radiologic features, with characteristic imaging seen only in a minority. Incorporation of additional features into current diagnostic criteria can identify the full spectrum of clinical presentations of AIP.",
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T2 - The Mayo Clinic Experience

AU - Chari, Suresh T

AU - Smyrk, Thomas Christopher

AU - Levy, Michael J.

AU - Topazian, Mark

AU - Takahashi, Naoki

AU - Zhang, Lizhi

AU - Clain, Jonathan E.

AU - Pearson, Randall K.

AU - Petersen, Bret Thomas

AU - Vege, Santhi Swaroop

AU - Farnell, Michael B.

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