Original article—liver, pancreas, and biliary tract
Prevalence, Diagnosis, and Profile of Autoimmune Pancreatitis Presenting with Features of Acute or Chronic Pancreatitis

https://doi.org/10.1016/j.cgh.2009.09.024Get rights and content

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 (Histology, Imaging features, Serology, Other organ involvement and Response to steroid treatment) 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.

Section snippets

Methods

The protocol was approved by the Mayo Clinic Institutional Review Board. We utilized a prospectively collected list of all patients presenting to the Mayo Pancreas Clinic for evaluation of acute or chronic pancreatitis between January 2005 and June 2006. We reviewed electronic medical records of all patients in this list to retrieve demographic, clinical, radiologic, and laboratory data and the final clinical diagnosis.

To investigate the clinical profile of AIP patients presenting with acute or

Prevalence of AIP Among Patients Evaluated for Acute or Chronic Pancreatitis

During the period January 2005 through June 2006, 178 patients (89 males [50%]) were evaluated at the Mayo Pancreas Clinic for possible acute or chronic pancreatitis. Of the 178 patients, the final clinical diagnosis was acute pancreatitis in 58 patients (32.6%), chronic pancreatitis in 79 patients (44.4%), benign or malignant tumor of the pancreas in 6 patients (3.4%), miscellaneous etiologies in 12 patients (6.7%), and no discernible disease of the pancreas in 16 patients (9%). Seven of 178

Discussion

Acute and chronic pancreatitis are considered to be uncommon clinical manifestations of AIP. In our study approximately 4% of patients evaluated for etiology of suspected pancreatitis had AIP based on HISORt criteria. We also reviewed the clinical profile of AIP patients diagnosed at our center from June 1999 through October 2008 to identify patients who had acute or chronic pancreatitis. We report that 24% of AIP patients had acute pancreatitis and 11% presented as chronic pancreatitis. In

References (19)

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    Type 2 AIP affects only the pancreas, seen in younger patients and is not associated with IgG4 elevation.93 Among 178 patients with autoimmune pancreatitis, >33% of them had features of AP or CP at the time of presentation.94 Although autoimmune pancreatitis is considered a form of CP, it has distinct clinical and histologic features and responds well to corticosteroid therapy.

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This article has an accompanying continuing medical education activity on page 3. Learning Objectives—Upon completion of this activity, the successful learner should be able to define the expected frequency, clinical features, and diagnostic criteria of autoimmune pancreatitis.

Conflict of interest The authors disclose no conflicts.

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