Clinical features and relapse rates after surgery in type 1 autoimmune pancreatitis differ from type 2: a study of 114 surgically treated European patients.
BACKGROUND
At the recent consensus conference on autoimmune pancreatitis (AIP) in Honolulu, we presented preliminary data from our study of surgically treated AIP patients. Our data strongly supported the separation of AIP into type 1 and type 2. Our study is based on a total of 114 surgically treated European AIP patients. Our aims were to elucidate serum IgG4 elevation, other organ involvement, relapse of disease, steroid treatment and diabetes after surgery in 114 surgically treated European AIP patients.


METHODS
88 pancreaticoduodenectomies, 22 left-sided resections and 4 total pancreatectomies were examined. All cases were graded for granulocytic epithelial lesions, IgG4-positive cells, storiform fibrosis, phlebitis and eosinophilic granulocytes. Follow-up data were obtained from 102/114 patients, mean follow-up was 5.3 years.


RESULTS
Histologically, 63 (55.3%) of the 114 patients fulfilled the criteria of type 1 AIP, while 51 (44.7%) patients fulfilled the criteria of type 2 AIP. Type 1 AIP patients were older and more often males than type 2 AIP patients. Elevation of serum IgG4, involvement of extrapancreatic organs, disease relapse, systemic steroid treatment and diabetes after surgery were noted more often in type 1 AIP, while inflammatory bowel disease (IBD) was observed mainly in type 2 AIP.


CONCLUSIONS
Histological typing of AIP is clinically important because type 1 AIP is part of the IgG4-related disease and type 2 AIP is associated with IBD. Our data also show that relapse of disease and steroid treatment after surgery occur more frequently in type 1 than in type 2 AIP.
Dept. of Pathology, Odense University Hospital, Odense, Denmark. S.Detlefsen@gmx.net
Sönke
S
Detlefsen
Giuseppe
G
Zamboni
Luca
L
Frulloni
Bernd
B
Feyerabend
Felix
F
Braun
Oke
O
Gerke
Anna Melissa
AM
Schlitter
Irene
I
Esposito
Günter
G
Klöppel
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000150:complications / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D015212:Inflammatory Bowel Diseases / Q000150:complications; D008297:Male; D008875:Middle Aged; D010179:Pancreas / Q000473:pathology; D016577:Pancreaticoduodenectomy; D010195:Pancreatitis / Q000145:classification / Q000473:pathology / Q000601:surgery*; D012008:Recurrence; D044465:White People
D007074:Immunoglobulin G