Highly active state of autoimmune pancreatitis with mikulicz disease

Pancreas. 2010 Jan;39(1):e6-10. doi: 10.1097/MPA.0b013e3181bc119d.

Abstract

Objective: Patients with autoimmune pancreatitis (AIP) sometimes present with Mikulicz disease (MD); however, the clinical features regarding these AIP patients with MD have not yet been fully elucidated. Our aim is to study the clinical differences between AIP with and without MD.

Methods: Twenty-eight AIP patients were divided into 2 groups, one with MD and one without it. The following factors having a possible association with the presence or absence of MD were investigated: sex; serum IgG and IgG4 levels; the presence or absence of antinuclear autoantibodies, jaundice, diabetes mellitus, swollen duodenal papilla, diffuse pancreatic swelling, spontaneous remission, and relapse.

Results: The MD and non-MD groups consisted of 5 AIP and 23 AIP patients, respectively. The results of univariate analysis revealed that AIP patients presenting with MD were significantly associated with a younger onset, female predominance, high serum IgG4 titer, and diffuse pancreatic swelling (P < 0.05). In 4 of the MD patients, onset preceded pancreatitis.

Conclusions: Autoimmune pancreatitis patients presenting with MD tended to have different clinical features from the non-MD AIP patients, such as having an earlier onset, female tendency, and diffuse pancreatic swelling with a high titer of serum IgG4. Autoimmune pancreatitis with MD tended to precede gastroenterological events.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age Factors
  • Aged
  • Antibodies, Antinuclear / blood
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / pathology*
  • Female
  • Humans
  • Immunoglobulin G / blood
  • Male
  • Middle Aged
  • Mikulicz' Disease / complications*
  • Pancreas / pathology
  • Pancreatitis / blood
  • Pancreatitis / complications
  • Pancreatitis / pathology*
  • Sex Factors

Substances

  • Antibodies, Antinuclear
  • Immunoglobulin G