IgG4-related disease presenting as hoarseness and postcricoid ulcer

Immunoglobulin G4–related disease (IgG4-RD) is an uncommon immune-mediated disorder with heterogeneous clinicopathologic features and variable disease manifestations.1 IgG4-RD is characterized by single-organ or multiorgan involvement, and tissue infiltration with IgG4 plasma cells and associated fibrosclerosis.2–5 Autoimmune pancreatitis represents the prototype of IgG4-RD, yet involvement of almost all organs has been reported.1–7 Elevated IgG4 serum concentration is noted in the majority of patients with IgG4-RD and is generally related to the more severe disease phenotype.

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