BIRC3

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BIRC3

A gene on chromosome 11q22 that belongs to the inhibitor of apoptosis (IAP) gene family, which encode negative regulatory proteins that prevent apoptotic cell death. BIRC3 acts by binding to tumour necrosis factor receptor-associated factors TRAF1 and TRAF2, interfering with activation of ICE-like proteases.
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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.
Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience

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