Activation of the IFN-α-IL-33 axis may contribute to the development of a specific type of tissue fibrosis, called storiform fibrosis, in type 1 AIP/IgG4-RD1–3 since the inhibition of this axis markedly decreased, not only chronic inflammation, but also pancreatic fibrosis in experimental AIP15,16,18. Here, IL33 is linked to autoimmune pancreatitis type 1.