Prevalence, Diagnosis, and Profile of Autoimmune Pancreatitis Presenting with Features of Acute or Chronic Pancreatitis

Published on Jan 1, 2010in Clinical Gastroenterology and Hepatology8.549
· DOI :10.1016/J.CGH.2009.09.024
Raghuwansh P. Sah20
Estimated H-index: 20
(Mayo Clinic),
Rahul Pannala21
Estimated H-index: 21
(Mayo Clinic)
+ 9 AuthorsSanthi Swaroop Vege50
Estimated H-index: 50
(Mayo Clinic)
Background & Aims Little is known about how many patients with features of acute pancreatitis (AP) or chronic pancreatitis (CP) have autoimmune pancreatitis (AIP); most information comes from case reports. We explored the clinical profiles and relationship between these diseases. Methods We evaluated 178 patients presenting to our Pancreas Clinic between January 2005 and June 2006 for evaluation of the etiology of their suspected pancreatitis; AIP was diagnosed when patients met HISORt ( H istology, I maging features, S erology, O ther organ involvement and R esponse to steroid t reatment) criteria. In a separate cohort of patients with AIP from our database, we identified patients who presented with features of AP (≥2 of abdominal pain, increased pancreatic enzymes, pancreatic inflammation determined by imaging analyses) or CP (≥1 of pancreatic calcification, irregular main pancreatic duct dilation, or marked atrophy) and determined their clinical profile. Results Only 7/178 (3.9%) patients evaluated for etiology of suspected pancreatitis had AIP. Among 63 AIP patients in our database, 22 (34.9%) had features of AP (n = 15) or CP (n = 7) at presentation (average age 53.4 ± 19.0 years, all males). Patients with AIP and pancreatitis were characterized by presence of obstructive jaundice (59.1%), increased levels of liver enzymes (81.8%), increased levels of serum immunoglobulin G4 (80.9%), and other organ involvement (69.1%). All 19 patients presenting with pancreatitis who were treated with steroids responded to treatment. Conclusions While AIP is an uncommon etiology for acute or chronic pancreatitis, >33% of AIP have features of acute or chronic pancreatitis at presentation.
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