Gut Liver.  2013 Jan;7(1):96-99.

Clinical Characteristics of Patients with Autoimmune Pancreatitis with or without Mikulicz's Disease and Mikulicz's Disease Alone

• Kuruma S
• Kamisawa T
• Tabata T
• Hara S
• Fujiwara T
• Kuwata G
• Egarashira H
• Koizumi K
• Setoguchi
• Fujiwara J
• Arakawa T
• Momma K
• Mitsuhashi T
• Sasaki T

Abstract

BACKGROUND/AIMS
The objective of this study was to compare the clinical characteristics of patients with autoimmune pancreatitis (AIP) with or without Mikulicz's disease (MD) and with MD alone.
METHODS
We investigated the clinical findings in 15 AIP patients with MD (group A+M), 49 AIP only patients (group A), and 14 MD only patients (group M).
RESULTS
The male-female ratio was significantly higher in group A+M (73%, p<0.05) and group A (78%, p<0.01) than group M (21%). Serum immunoglobulin G (IgG) levels were significantly higher in group A+M than in group A (p<0.01) and group M (p<0.05). Serum IgG4 levels were significantly higher in group A+M than in group A (p<0.01). Other organ involvement was observed in 73% (11/15) of patients in group A+M. The number of patients with diabetes mellitus was significantly higher in group A+M (66%, p<0.01) and group A (51%, p<0.05) than in group M (7%). All of the patients responded well to steroid therapy, but the relapse rate in group A+M (33%) was significantly higher than that in group A (3%, p<0.01). Salivary gland function was impaired in all groups compared with the control group, but the degree of dysfunction was less in group A compared with group A+M and group M.
CONCLUSIONS
The relapse rate of AIP in MD patients was significantly higher than that of AIP in patients without MD.

Keyword

Autoimmune pancreatitis; Mikulicz' disease; Immunoglobulin G; IgG4-related disease; Steroid therapy

MeSH Terms

Diabetes Mellitus
Humans
Immunoglobulin G
Mikulicz' Disease
Pancreatitis
Recurrence
Salivary Glands
Immunoglobulin G