Abstract
BackgroundImmunoglobulin G4 related disease (IgG4-RD) is a poorly understood chronic inflammatory disorder affecting the middle-aged and elderly that can present to the otolaryngologist. We aim to summarize the current literature regarding the manifestations and management of IgG4-RD in the head and neck.MethodsPubmed and EMBASE were searched using the term relevant search algorithm utilizing keywords such as: IgG4 related disease, head and neck, orbit, salivary glands, sialadenitis, Kuttner, angiocentric eosinophilic fibrosis, submandibular, lacrimal, thyroid, dacryoadenitis, nasal, sinus, and Mikulicz’s. Reference lists were searched for identification of relevant studies.Case reports, original research and review articles published in English from 1964 to 2014 whose major topic was IgG4-RD affecting the head and neck were included. Data regarding patient demographics, presentation, histopathology, management and treatment outcomes of IgG4-RD were extracted. Level of evidence was also assessed and data were pooled where possible. Three independent reviewers screened eligible studies; extracted relevant data and discrepancies were resolved by consensus, where applicable. Descriptive and comparative statistics were performed.ResultsFourty-three articles met our inclusion criteria. IgG4-RD most often presents as a mass lesion in the head and neck region. Common diagnostic features include: 1) elevated serum IgG4 level, 2) marked infiltration of exocrine glands by IgG4-positive plasma cells with fibrosis, and 3) marked improvement with corticosteroid therapy and additional immunosuppressive therapy in corticosteroid refractory cases. Early diagnosis and involvement of rheumatology is important in management.ConclusionsIgG4-RD is a challenging non-surgical disease that has multiple manifestations in the head and neck. It must be distinguished from various mimics including malignancy, systemic diseases, and infectious. Otolaryngology-Head and Neck surgeons should be aware of this condition and its management.
Highlights
Immunoglobulin G4 – related disease (IgG4-RD) is a newly described fibroinflammatory condition that often presents as a tumefactive lesion that can affect nearly every organ system
Immunoglobulin G4 related disease (IgG4-RD) was first recognized after a connection between elevated serum IgG4 levels and inflammatory mass lesions in the pancreas causing autoimmune pancreatitis was made by Hamano et al in 2001 [1]
This study aims to examine the various presentations of IgG4-RD in the head and neck, and present the management and outcomes reported in the literature
Summary
Immunoglobulin G4 – related disease (IgG4-RD) is a newly described fibroinflammatory condition that often presents as a tumefactive lesion that can affect nearly every organ system. It is postulated that the inflammatory and fibrotic processes that drives IgG4-RD are propagated by a combination of Th2 cells and regulatory T cells (Treg cells) [4]. This is contrary to most autoimmune disorders where. The exact histological findings vary greatly depending on the tissue affected and clinical presentation. Immunoglobulin G4 related disease (IgG4-RD) is a poorly understood chronic inflammatory disorder affecting the middle-aged and elderly that can present to the otolaryngologist. We aim to summarize the current literature regarding the manifestations and management of IgG4-RD in the head and neck
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