Immunoglobulin G4-related diseases in the head and neck: A systematic review

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.

Zaburzenia tarczycy związane z immunoglobuliną G4 — wyzwanie diagnostyczne i wyniki kliniczne

“…However, in contrast to the female preponderance of autoimmune (Hashimoto's) thyroiditis, there is no sex predilection for IgG4RTD. It is believed to occur equally among males and females [1,2]. Recent reports have also suggested that a small fraction of Graves' disease patients also have elevated IgG4 levels with some features suggestive of IgG4TD [21,22].…”

“…IgG4-related disease per se as an entity was recognised only in 2001, with autoimmune pancreatitis being the prototype organ involvement [1]. Thyroid involvement in IgG4RD is rare, believed to occur in < 4% of cases [1,2]. However, it is likely that the diagnosis is grossly under reported due …”

“…In the head and neck region, orbits are the most commonly involved organs observed in more than 50% of cases, followed by salivary glands (22%) and lacrimal glands (18.6%) [2]. Thyroid involvement is a rare manifestation of IgG4RD, believed to occur in 4% of patients [2].…”

“…The head and neck region is the part of the body that is perhaps most commonly involved in IgG4RD [2]. In the head and neck region, orbits are the most commonly involved organs observed in more than 50% of cases, followed by salivary glands (22%) and lacrimal glands (18.6%) [2].…”

“…The Japanese guidelines recommend the use of prednisolone at 0.6 mg/kg/day (usually at 30-40 mg per day) for a period of three months, which is known as the induction regimen for inducing remission, followed by a maintenance therapy of low dose prednisolone (2.5-5 mg per day) for 6-12 months, to maintain remission and prevent relapse [11]. Steroid sparing agents like methotrexate, azathioprine, mycophenolate, rituximab, cyclosporine, chlorambucil, and cyclophosphamide have been tried in the management of IgG4RD [2,11]. However, their use in the management of IgG4RTD needs to be evaluated.…”