Keiko Ohno1, Yoko Matsuda2, Tomio Arai2, Takahiko Sugihara3, Shoko Iga3, Yurika Kimura4. 1. Department of Otorhinolaryngology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan Department of Otolaryngology, Graduate School, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan takoto@tmd.ac.jp. 2. Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Itabashi-ku, Tokyo, Japan. 3. Department of Medicine and Rheumatology, Tokyo Metropolitan Geriatric Hospital, Itabashi-ku, Tokyo, Japan. 4. Department of Otorhinolaryngology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan Department of Otolaryngology, School of Medicine, Showa University, Shinagawa-ku, Tokyo, Japan.
Abstract
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.
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.