Autoimmune pancreatitis: differences between the focal and diffuse forms in 87 patients.
OBJECTIVE
Autoimmune pancreatitis (AIP) is a particular type of chronic pancreatitis that can be classified into diffuse and focal forms. The aim of this study was to analyze clinical and instrumental features of patients suffering from the diffuse and focal forms of AIP.


METHODS
AIP patients diagnosed between 1995-2008 were studied.


RESULTS
A total of 87 AIP patients (54 male and 33 female patients, mean age 43.4+/-15.3 years) were studied. Focal-type AIP was diagnosed in 63% and diffuse-type in 37%. Association with autoimmune diseases was observed in 53% of cases, the most common being ulcerative colitis (30%). Serum levels of IgG4 exceeded the upper normal limits (135 mg/dl) in 66% of focal AIP and in 27% of diffuse AIP (P=0.006). All patients responded to steroids. At recurrence non-steroid immunosuppressive drugs were successfully used in six patients. Recurrences were observed in 25% of cases, and were more frequent in focal AIP (33%) than in diffuse AIP (12%) (P=0.043), in smokers than in non-smokers (41% vs. 15%; P=0.011), and in patients with pathological serum levels of IgG4 compared to those with normal serum levels (50% vs. 12%; P=0.009). In all, 23% of the patients underwent pancreatic resections. Among patients with focal AIP, recurrences were observed in 30% of operated and in 34% of not operated patients.


CONCLUSIONS
Focal-type and diffuse-type AIP differ as regards clinical symptoms and signs. Recurrences occur more frequently in focal AIP than in diffuse AIP. The use of non-steroid immunosuppressants may be a therapeutic option in relapsing AIP.
Frulloni
Luca
L
Department of Biomedical and Surgical Sciences, University of Verona, Verona, Italy. luca.frulloni@univr.it
Scattolini
Chiara
C
Falconi
Massimo
M
Zamboni
Giuseppe
G
Capelli
Paola
P
Manfredi
Riccardo
R
Graziani
Rossella
R
D'Onofrio
Mirko
M
Katsotourchi
Anna Maria
AM
Amodio
Antonio
A
Benini
Luigi
L
Vantini
Italo
I
D000328:Adult; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D050500:Pancreatitis, Chronic / Q000188:drug therapy / Q000276:immunology*; D012008:Recurrence; D016019:Survival Analysis
D007166:Immunosuppressive Agents