1. Autoimmune pancreatitis
Esmee Burgers
Louise Dugué
Supervized by Dr. habil. Rakonczay Zoltan

2. Outline Introduction Types of AIP Pathogenesis Genetic predispositions
Inflammatory mechanisms
Treatment
Take home messages

3. introduction Rare version of chronic pancreatitis Clinically
Incidence: 1 in ~ people (Japan)
Clinically
Mild or no abdominal pain
Jaundice
Cachexia
Differential diagnosis
Pancreatic cancer

4. Two types of aip Type 1 Type 2 Nomenclature IgG4 related
Type 1
Type 2
Nomenclature
IgG4 related
AIP with GELs, IgG4 unrelated
Prevalence
Asia > US > Europe
Europe > US > Asia
Age
Older than 60
Older than 40
Sex
Male > female
No sex bias
Abdominal Pain
Very rare
Rare
Steroid therapy
Responsive
Relapse
High rate
Extra-pancreatic lesions
IgG4-related disease
Inflammatory bowel disease
Complications
Frequent
Uncommon

5. Histology Obliterative phlebitis Storiform fibrosis
Hart P et al. Gastroenterology 2015;149:39-51

6. Histology Immunohistochemistry for IgG4
Zhang L et al. Modern pathology 2007;20:23-28

7. Histology
GEL
Zhang L et al. Modern pathology 2007;20:23-28

8. Pathogenic mechanisms1
Carbonic anhydrase!!
Modified from Okazaki K, Uchida K. Autoimmune pancreatitis, the past, present and future. Pancreas 2015; 44:

9. Genetic predispositions
HLA
DQβ1 and DRβ1 : MHC II genes
DQβ1*0401 and DRβ1*0405 : predicting factors
DQβ1 57 mutation  relapses
PRSS1
Cationic trypsinogen enzyme  acute pancreatitis
Low rate of relapses

10. Genetic predispositions
MST1
Serine/threonine kinase
Traffic of immune cells
Extra-pancreatic lesions
CTLA-4
Maintenance of tolerance
Expressed on CD4+ and CD8+ T cells
Those cells infiltrate pancreatic tissue
Rat study  CD4+ T cells can induce pancreatitis
Negative regulator of T-cells

11. Inflammatory mechanisms
Hart P, Zen Y and Chari S. Recent advances in autoimmune pancreatitis. Gastroenterology 2015;149:39-51

12. treatment TYPE 1 AIP TYPE 2 AIP Response to steroids Positive
Other treatments possible
Immunomodulators Rituximab
Relapse
Common
Rare

13. Take home messages AIP is curable – responsive to corticosteroids
2 types
Type 1  IgG4
Type 2  GELs
Differentiation based on histology
Inflammatory process
Innate immune system
IgG-4
Th and Tregs

14. Thank you for your attention!
Any questions?