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Highly active state of autoimmune pancreatitis with mikulicz disease
Kensuke Kubota1, Tomoko Wada, Shingo Kato
1Division of Gastroenterology,Yokohama City University, Graduate School of Medicine, Yokohama, Japan. kubotak@yokohama-cu.ac.jp
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.
Insights
Autoimmune pancreatitis (AIP) patients with Mikulicz disease (MD) often present with earlier onset, female predominance, and diffuse pancreatic swelling. High serum IgG4 levels are common in AIP with MD, which may precede other symptoms.
Area of Science:
- Gastroenterology
- Immunology
- Pancreatology
Background:
- Autoimmune pancreatitis (AIP) is a chronic inflammatory condition.
- Mikulicz disease (MD) can be associated with AIP.
- Clinical distinctions between AIP with and without MD require further investigation.
Purpose of the Study:
- To investigate the clinical differences between AIP patients with and without MD.
- To identify factors associated with the presence of MD in AIP patients.
Main Methods:
- A cohort of 28 AIP patients was divided into two groups: those with MD and those without.
- Clinical data, including demographics, serum IgG and IgG4 levels, autoantibodies, and specific clinical manifestations (jaundice, diabetes, papilla swelling, pancreatic swelling, remission, relapse), were analyzed.
- Univariate analysis was employed to identify significant associations.
Main Results:
- The study included 5 AIP patients with MD and 23 without.
- AIP patients with MD showed a significant association with younger age of onset, female predominance, elevated serum IgG4 levels, and diffuse pancreatic swelling.
- In a subset of MD patients, the onset of MD preceded the diagnosis of AIP.
Conclusions:
- AIP patients with MD exhibit distinct clinical features compared to those without MD, including earlier onset, female tendency, diffuse pancreatic swelling, and high serum IgG4 titers.
- MD may precede the development of AIP in some cases, suggesting a potential link in the disease progression.
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