A unique profile of serum cytokines in type 1 autoimmune pancreatitis and chronic rhinosinusitis

Abstract

Background: Type 1 autoimmune pancreatitis (AIP) is a pancreatic manifestation of IgG4-related disease (IgG4-RD). Although AIP and IgG4-RD are characterized by multiple organ involvement including salivary glands, lung, and kidney, co-occurrence of chronic rhinosinusitis (CRS) and AIP/IgG4-RD has been poorly defined. Objective: We explored molecular mechanism accounting for the co-occurrence of CRS and AIP/IgG4-RD. Methods: Serum concentrations of IFN-α and IL-33 were measured by enzyme-linked immune-sorbent assay. Results: We encountered a patient with concurrent type 1 AIP/IgG4-RD and CRS. Induction of remission by prednisolone (PSL) for type 1 AIP/IgG4-RD led to a marked improvement of CRS. Serum cytokine analysis after PSL treatment revealed a marked reduction in serum concentrations of IFN-α and IL-33, both of which are candidate pathogenic cytokines for AIP/IgG4-RD. Conclusions: Given that IL-33 is shared as one of pathogenic cytokines by type 1 AIP/IgG4-RD and CRS, enhanced IL-33 responses may cause concurrent type 1 AIP/IgG4-RD and CRS.