In contrast to most studies of the eastern hemisphere, in which up to 90% of the patients suffered from AIP1, we found an almost equal distribution of AIP1 and AIP2 patients (55.6% versus 44.4%) in our cohort.
Compared to other systemic autoimmune diseases like lupus erythematosus, ANA screening with a cut-off greater than 1: 80 revealed that 5 of our AIP1 patients and 7 of the AIP2 patients were ANA-positive showing higher titers by trend in the AIP2 group.
According to current literature, AIP1 is less likely to be associated with UC than AIP2 [8, 9].
The efficacy of steroid therapy in the treatment of AIP1 and AIP2 has been established by the demonstration of short-term serological (initial response rates > 90%) and radiological responses as well as clinical improvement in several studies.
Interestingly, in our cohort with a cumulative survey time of 1020 months in total, 3 of 20 AIP1 patients (15%) developed cholangio-cellular carcinoma (CCC) and one of these patients was diagnosed synchronously with AIP.
In contrast to most studies in Asia in which up to 90% of the patients suffered from AIP1, we noticed a high rate of AIP2 patients in our cohort in Germany with 44.4%.
Abbreviations AIC: Autoimmune cholangitis AIP: Autoimmune pancreatitis AIP1: Autoimmune pancreatitis type 1 AIP2: Autoimmune pancreatitis type 2 AP: Alkaline phosphatase CCC: Cholangiocellular carcinoma EUS: Endoscopic ultrasound-guided sonography GELs: Granulocytic epithelial lesions [gamma]-GT: [gamma]-Glutamyltransferase HCC: Hepatocellular carcinoma HLA: Human leukocyte antigen IBD: Inflammatory bowel disease IDCP: Idiopathic duct centric pancreatitis IgG4: Immunoglobulin G4 LPSP: Lymphoplasmacytic sclerosing pancreatitis MMF: Mycophenolate mofetil UC: Ulcerative colitis.
Caption: FIGURE 3: Cholestatic liver enzymes in patients with AIP1 and AIP2.
Caption: FIGURE 4: Serum levels of IgG4 are significantly increased in patients with AIP1.
Caption: FIGURE 5: Patients with AIP1 and AIP2 revealed increased ANA titers.