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Research Article: Localized lymphoid hyperplasia with prominent IgG4-positive plasma cells in the inferior turbinate mimicking IgG4-related disease

Date Published: 2026-10-14

Abstract:
Localized lymphoid masses of the inferior turbinate with prominent IgG4-positive plasma cell infiltration are occasionally encountered in clinical practice, yet their nature and relationship to IgG4-related disease (IgG4-RD) remain uncertain. This study aimed to characterize the clinical, histopathological, and therapeutic features of these lesions. We retrospectively reviewed patients presenting with localized submucosal masses of the inferior turbinate identified on nasal endoscopy at Seoul National University Hospital between January 2020 and May 2023. Biopsies were performed in cases with mucosal disruption, including crusting or bleeding. Classification of IgG4-RD was based on the 2019 American College of Rheumatology/European League Against Rheumatism criteria. The 2011 Comprehensive diagnosis criteria were applied to the patients with available biopsies. Of 14,103 patients undergoing nasal endoscopy, 41 (0.3%) demonstrated localized inferior turbinate submucosal lesions. Eighteen patients underwent biopsy, all of which revealed dense lymphoplasmacytic infiltration with variable IgG4-positive plasma cell counts. An IgG4+/IgG+ plasma cell ratio exceeding 40% was observed in 10 cases (55.6%). However, characteristic histopathological features of IgG4-RD, including storiform fibrosis and obliterative phlebitis, were absent. Only two patients fulfilled the 2019 classification criteria for IgG4-RD, both of whom had extra-nasal involvement. According to the 2011 Comprehensive diagnosis criteria, 2 patients were classified as definite IgG4-RD. Spontaneous improvement during observation occurred in a minority of patients. Surgical treatment resulted in durable symptom control with minimal recurrence, whereas corticosteroid therapy showed variable effectiveness. Localized IgG4-rich lymphoid hyperplasia of the inferior turbinate may represent a distinct benign inflammatory lesion, mimicking a sinonasal manifestation of IgG4-RD. Further studies are required to clarify its pathophysiology, optimal management, and long-term outcomes.

Introduction:
IgG4-related disease (IgG4-RD) is a rare, multi-system fibro-inflammatory condition characterized by dense lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, tissue infiltration by IgG4-positive plasma cells, and frequently elevated serum IgG4 concentrations ( 1 – 3 ). In the head and neck region, IgG4-RD most commonly affects the lacrimal, orbital, parotid, and submandibular glands, as well as the thyroid gland and cervical lymph nodes. In contrast, sinonasal involvement is uncommon ( 4…

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