Sinonasal Presentations of Immunoglobulin G4-Related Disease: A Scoping Review Publisher



Farrokh F¹ ; Rimmer RA² Show Affiliations
Source: Laryngoscope Published:2025

Abstract

Objective: Immunoglobulin G4 related disease (IgG4RD) is a recently identified systematic immune disease that may manifest in various organ systems. The latest classification criteria from 2019 combine data from 11 “typical” organ systems for diagnosis, notably excluding the sinonasal region. As sinonasal involvement is not included in this criteria, we performed a scoping review to better clarify sinonasal presentations of IgG4RD. Data Sources: We conducted a literature search in three bibliographic resources: Pubmed, Scopus, and Web of science. Review Methods: All the databases' results were reviewed by two authors utilizing Endnote and Rayyan platforms. Articles were included if they presented a case (or cases) with sinonasal IgG4RD. Critical appraisal of all included articles was performed according to JBI guidelines. Results: Eighty articles containing 145 patients with sinonasal manifestations of IgG4RD were included. Ages ranged from 13 to 83 years (mean 54.57 ± 15.85 years). The most common clinical symptoms were nasal (63.4%) and visual/orbital (37.9%) complaints. The most frequent sites of involvement were maxillary sinus (N = 75), ethmoid sinus (N = 65) and nasal cavity (N = 59). Seventy-three percent of patients had involvement of at least one other organ system outside the sinonasal region. Conclusion: Sinonasal manifestations have been described despite not being considered a typical organ system eligible for the recent classification criteria of IgG4RD; however, simultaneous involvement of other organ systems outside the sinonasal region is common. Familiarity with common presentations of IgG4RD and related diagnostic elements is critical to the diagnosis and management of all involved sites. Level of Evidence: N/A. © 2025 The American Laryngological, Rhinological and Otological Society, Inc.