E L Culver1, A Hunt2, E Crewe2, K A Shah3, P Martinez-Devesa2. 1. Translational Gastroenterology Unit,John Radcliffe Hospital and Oxford University,Oxford,UK. 2. Department of Otorhinolaryngology - Head and Neck Surgery,John Radcliffe Hospital,Oxford,UK. 3. Department of Cellular Pathology,John Radcliffe Hospital,Oxford,UK.
Abstract
BACKGROUND: ENT surgeons may be the first specialists to encounter and diagnose patients with salivary gland disease. A new entity involving the salivary glands has recently been described of which ENT surgeons need to be aware: immunoglobulin G4 related chronic sclerosing sialadenitis. METHOD: A literature search of Medline, Embase and Cochrane Library databases was performed, using the search terms 'IgG4', 'hyperIgG4 syndrome' and 'IgG4 related chronic sclerosing sialadenitis'. RESULTS: Knowledge concerning immunoglobulin G4 related chronic sclerosing sialadenitis is rapidly increasing. This new entity is part of a fibro-inflammatory corticosteroid-responsive systemic disease (immunoglobulin G4 related disease) and has been described in almost every organ. Biopsy of the submandibular gland can be diagnostic. However, the diagnosis can easily be overlooked if: clinical suspicion is not high, one is unaware of the classical morphology and/or immunoglobulin G4 staining is not performed. This paper presents a summary of the current understanding of the disease and its management. CONCLUSION: ENT surgeons should be aware of this new disease entity. Patients with systemic disease should be managed under a multidisciplinary team, with input from clinicians who have an interest in such diseases (such as gastroenterologists and rheumatologists), and input from histopathologists and radiologists.
BACKGROUND: ENT surgeons may be the first specialists to encounter and diagnose patients with salivary gland disease. A new entity involving the salivary glands has recently been described of which ENT surgeons need to be aware: immunoglobulin G4 related chronic sclerosing sialadenitis. METHOD: A literature search of Medline, Embase and Cochrane Library databases was performed, using the search terms 'IgG4', 'hyperIgG4 syndrome' and 'IgG4 related chronic sclerosing sialadenitis'. RESULTS: Knowledge concerning immunoglobulin G4 related chronic sclerosing sialadenitis is rapidly increasing. This new entity is part of a fibro-inflammatory corticosteroid-responsive systemic disease (immunoglobulin G4 related disease) and has been described in almost every organ. Biopsy of the submandibular gland can be diagnostic. However, the diagnosis can easily be overlooked if: clinical suspicion is not high, one is unaware of the classical morphology and/or immunoglobulin G4 staining is not performed. This paper presents a summary of the current understanding of the disease and its management. CONCLUSION: ENT surgeons should be aware of this new disease entity. Patients with systemic disease should be managed under a multidisciplinary team, with input from clinicians who have an interest in such diseases (such as gastroenterologists and rheumatologists), and input from histopathologists and radiologists.
Authors: George Goodchild; Rory Jr Peters; Tamsin N Cargill; Harry Martin; Adetokunbo Fadipe; Maria Leandro; Adam Bailey; Jane Collier; Louisa Firmin; Manil Chouhan; Manuel Rodriguez-Justo; Ross Sadler; Roger W Chapman; Helen Bungay; Eve Fryer; Joel David; Raashid Luqmani; Eleanor Barnes; George J Webster; Emma L Culver Journal: Clin Med (Lond) Date: 2020-05 Impact factor: 2.659