Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Positive Otitis Media and Rhinosinusitis With Pathological Features of Immunoglobulin G4-Related Disease: A Case Report

Background:

Immunoglobulin G4-related disease (IgG4-RD) and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have different clinical and pathological features. However, differentiation between these 2 disorders is sometimes difficult.

Objective:

To report a case involving a patient with characteristics of both IgG4-RD and AAV.

Methods:

Case report with literature review.

Results:

We report a case of myeloperoxidase-ANCA–positive otitis media and rhinosinusitis with pathological features of IgG4-RD in a 73-year-old man. The patient was first clinically suspected to have granulomatosis with polyangiitis. All of the main characteristic pathological features of IgG4-RD were present: dense lymphoplasmacytic infiltration, increased numbers of IgG4-positive plasma cells, storiform-type fibrosis, and obliterative phlebitis.

Conclusions:

The simultaneous presence of the characteristics of both IgG4-RD and AAV makes diagnosis and treatment difficult.

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