Recent advances in knowledge regarding the head and neck manifestations of IgG4-related disease

Abstract

IgG4-related disease (IgG4-RD) is a chronic inflammatory disorder, characterized by elevated serum IgG4 levels as well as abundant infiltration of IgG4-positive plasmacytes and fibrosis in various organs, including the head and neck region. In particular, the salivary glands, orbit, and thyroid are common sites of disease involvement. IgG4-RD is diagnosed based on various clinical, serological, and histopathological findings, none of which are pathognomonic. Hence, various differential diagnoses, which exhibit elevated serum IgG4 levels and infiltration of IgG4-postive cells into tissues, need to be excluded, especially malignant diseases and mimicking disorders. Systemic corticosteroids are generally effective in inducing IgG4-RD remission; however, recurrent or refractory cases are common. In addition, although the pathogenic mechanisms of IgG4-RD remain unclear, an antigen-driven inflammatory condition is believed to be involved. Recent studies have indicated the important pathogenic role of B cell/T cell collaboration and innate immunity in this disease. Nevertheless, additional research and discussions are needed to resolve many remaining questions. In this review, we provide an overview of the recent insights on the history, clinical features, diagnosis, and treatment of IgG4-RD in the head and neck region. Furthermore, we have also addressed the pathogenesis of this disease.

Clinical utility of 18F‐fluorodeoxyglucose/positron emission tomography in diagnosis of immunoglobulin G4–related sclerosing sialadenitis

“…In addition, patients with abundant IgG4‐positive plasma cell infiltration into the labial salivary gland tissue have a tendency to show FDG uptake by the PG . Therefore, relationships between increased uptake of FDG by the PG and IgG4‐SS disease activity in all major and minor salivary glands are considered to be present . In the present study, FDG uptake by the PG was observed in 35% patients, and their serum IgG4 levels and sIL‐2R were significantly higher (data not shown).…”

“…Immunoglobulin G4‐related disease (IgG4‐RD) is a systemic immune‐mediated disease characterized by elevated serum immunoglobulin G4 (IgG4) levels, dense lymphoplasmacytic infiltration, and fibrosis of the affected organs . IgG4‐RD, which is more common than previously suspected, can affect various organs, but the pathogenesis remains unclear.…”

“…IgG4‐RD, which is more common than previously suspected, can affect various organs, but the pathogenesis remains unclear. IgG4‐RD involving the head and neck is a common manifestation of this disease, affecting the salivary glands, lacrimal glands, sinonasal region, thyroid, pituitary gland, dura mater, and lymph nodes . In particular, IgG4‐related sclerosing sialadenitis (IgG4‐SS), which represents an enlargement of the salivary gland, is one of the most common lesions of IgG4‐RD .…”

Eosinophilic angiocentric fibrosis : a sino-orbital masquerader

“…Histopathology is the hallmark for diagnosis of IgG4 RD and subsequently EAF 10. It is imperative not to rely on measurement of serum IgG4 as it is not specific for IgG4 RD and has a low positive predictive value 11.…”

Correlation of hormonal and cytokines regulation in case of autoimmune thyroiditis

“…Лечение гипотиреоза на фоне АИТ сводится в основном к использованию заместительной тера-пии тиреоидными гормонами (ТГ) в случае его мани-фестации [4,7]. Однако по современным представле-ниям ТГ отводят роль антигенов в патогенезе ауто-иммунного воспаления ЩЖ [5,8,9], из чего следует, что заместительная терапия в этих случаях может поддерживать персистенцию аутоиммунного про-цесса, в том числе при IgG4-обусловленном тирео-идите, когда наиболее важное значение приобретает взаимодействие В-и Т-клеток с клетками врожден-ного иммунитета [1,4,5,10]. АИT относится к за-болеваниям, при котором аутоиммунное воспаление опосредовано прежде всего Т-клетками, а точная роль В-клеток в его патогенезе также остается до сих пор неясной [2][3][4].…”