Korean J Gastroenterol.  2008 Oct;52(4):230-246.

Clinical Characteristics, Recurrence Features, and Treatment Outcomes of 55 Patients with Autoimmune Pancreatitis

• Park SJ
• Kim MH
• Moon SH
• Han JH
• Park DH
• Lee SS
• Seo DW
• Lee SK

Abstract

BACKGROUND/AIMS: The purpose of this study was to assess the clinical characteristics, recurrence features, and treatment results of patients with autoimmune pancreatitis (AIP) and to determine the clinical predictive factors associated with recurrence.
METHODS
We analyzed the clinical, radiologic, laboratory, and recurrence features. We also evaluated treatment methods and outcomes, and clinical predictive factors associated with recurrence in 55 patients with AIP.
RESULTS
AIP may be misdiagnosed as pancreatic cancer due to the following characteristic features: (1) clinical findings similar to those of pancreatic cancer including weight loss (60.0%), obstructive jaundice (54.5%), and recent-onset diabetes (29.1%) as the major symptoms; (2) a preponderance in elderly men (mean, 57.7 years old; male, 81.8%); (3) pancreatic mass in computer tomography (21.8%). Serum IgG/IgG4 was elevated in 67.4% of cases. Other organ involvements were noted in 43.6% of cases. All patients (52/52) received steroid treatment have shown complete resolution or marked improvement in the presenting manifestations for which steroids were instituted. After median observation period of 32.8 (1-106) months, 9 patients (3-year cumulative recurrence rate, 20.0%) recurred. There was no significant clinical predictive factor for the recurrence of AIP. However, elevated serum IgG4 preceded recurrence in all patients whose serum IgG4 levels were checked at recurrence.
CONCLUSIONS
It is reasonable to understand AIP as a pancreatic lesion reflecting systemic disease, so called 'IgG4-related fibroinflammatory disease'. Steroid trial may be a practical diagnostic tool and a therapeutic one. Recurrence was not uncommon after the steroid treatment and serum IgG4 could be a monitoring marker for the recurrence in clinical practice.

Keyword

Autoimmune; Chronic pancreatitis; IgG4; Recurrence; Follow-up

MeSH Terms

Adolescent
Adult
Aged
Anti-Inflammatory Agents/therapeutic use
Autoimmune Diseases/*diagnosis/therapy
Cholangiopancreatography, Endoscopic Retrograde
Female
Humans
Immunoglobulin G/blood
Magnetic Resonance Imaging
Male
Middle Aged
Pancreatectomy
Pancreatitis, Chronic/*diagnosis/pathology/therapy
Predictive Value of Tests
Prednisolone/therapeutic use
Recurrence
Tomography, X-Ray Computed
Treatment Outcome