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Reumatologia 2020According to a new concept for the classification and division of autoimmune diseases, Mikulicz's disease and Küttner's tumor belong to immunoglobulin G4-related... (Review)
Review
According to a new concept for the classification and division of autoimmune diseases, Mikulicz's disease and Küttner's tumor belong to immunoglobulin G4-related diseases (IgG4-RD) and fulfil their diagnostic criteria. The aim of this study was to summarize the new classification concepts of IgG4-RD in the head and neck area and to review their clinical, histopathological and serologic criteria and the methods used in the diagnostic workup with respect to their advantages, limitations and differentiative value. The PubMed, Web of Science, Google Scholar, and Scopus databases were searched for articles published between 2009 and 2019 using the following key words: IgG4-related diseases, Mikulicz's disease, Küttner's tumor, salivary glands, xerostomia. Results of the review of the literature revealed that Mikulicz's disease and Küttner's tumor fulfil the same diagnostic criteria but may manifest different clinical symptoms which determine the choice of the different diagnostic tools.
PubMed: 32921832
DOI: 10.5114/reum.2020.98437 -
Japanese Journal of Ophthalmology Jan 2015Immunoglobulin G4 (IgG4)-related disease is a novel clinical entity characterized by infiltration of IgG4-immunopositive plasmacytes and elevated serum IgG4... (Review)
Review
Immunoglobulin G4 (IgG4)-related disease is a novel clinical entity characterized by infiltration of IgG4-immunopositive plasmacytes and elevated serum IgG4 concentration accompanied by enlargement of and masses in various organs, including the lacrimal gland, salivary gland, and pancreas. Recent studies have clarified that conditions previously diagnosed as Mikulicz disease as well as various types of lymphoplasmacytic infiltrative disorders of the ocular adnexa are consistent with a diagnosis of IgG4-related disease. Against this background, the diagnostic criteria for IgG4-related ophthalmic disease have recently been established, based on both the clinical and the histopathologic features of the ocular lesions. This article reviews these new criteria with reference to the comprehensive diagnostic criteria for IgG4-related disease for all systemic conditions reported in 2012.
Topics: Humans; Immunoglobulin G; Lacrimal Apparatus Diseases; Mikulicz' Disease; Orbital Pseudotumor; Plasma Cells
PubMed: 25392273
DOI: 10.1007/s10384-014-0352-2 -
Cells Feb 2023Diverse immune cell subsets have been described in IgG4-related disease (IgG4-RD). If there is a different immunophenotype according to clinical phenotype and activity...
Diverse immune cell subsets have been described in IgG4-related disease (IgG4-RD). If there is a different immunophenotype according to clinical phenotype and activity status is not known. Levels of IL-4-, IL-13-, IL-5-, and IL-21-producing CD4 T cells (Th2 subsets), CD4 cytotoxic T lymphocytes (CD4CTLs), T helper 9 cells, T follicular helper cells (Tfh; Tfh1/Tfh2/Tfh17/Tf regulatory [Tfr]), Foxp3 regulatory T cells, Type 1 regulatory T cells (Tr1), T helper 3 regulatory cells (Th3), IL-10-producing regulatory B cells (Bregs), IL-10-expressing regulatory plasmacytoid dendritic (pDC IL-10) cells, and M1 and M2 monocytes were determined by flow cytometry in 43 IgG4-RD patients and 12 controls. All immune subsets were higher in patients vs. controls. CD4/IL-4, CD4/IL-5, CD4CTLs, Tfh2, Tfh17, Tfr, and M1 monocyte cell number was different among IgG4-RD clinical phenotypes. The pancreato-hepato-biliary phenotype was characterized by a higher CD4CTLs, Tfh17, Tfh2, and Tfr and lower M1 cell number. An increased CD4CTLs and Th3 cell number distinguished the head and neck-limited phenotype, while the retroperitoneal/aortic and Mikulicz/systemic phenotypes were characterized by increased Th2 subsets. Tfh17, Tr1, Th3, pDC, M1, and M2 monocytes were augmented in active patients. In summary, the clinical heterogeneity of IgG4-RD might be driven by the participation of different immunophenotypes and, consequently, by a different fibroinflammatory process.
Topics: Humans; Interleukin-10; Immunoglobulin G4-Related Disease; Interleukin-4; Interleukin-5; Phenotype
PubMed: 36831337
DOI: 10.3390/cells12040670 -
Otolaryngologic Clinics of North America Jun 2021IgG4-related disease is a rare, immune-mediated, systemic disease that is characterized by soft tissue lymphocyte infiltration and resultant fibrosis. The salivary... (Review)
Review
IgG4-related disease is a rare, immune-mediated, systemic disease that is characterized by soft tissue lymphocyte infiltration and resultant fibrosis. The salivary glands are among the most commonly affected organs. Patients present with subacute submandibular and/or parotid swelling and sialadenitis. Diagnosis incorporates clinical, serologic, radiologic, and pathologic findings. Most cases respond quickly to systemic glucocorticoids. IgG4-related disease mimics many infectious, inflammatory, and neoplastic diseases. Therefore, IgG4-related disease is frequently misdiagnosed. A knowledge of the pathophysiology, diagnosis, and management of IgG4-related disease is important for providers who treat salivary gland diseases.
Topics: Glucocorticoids; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Salivary Glands; Sialadenitis
PubMed: 34024478
DOI: 10.1016/j.otc.2021.02.002 -
Reumatologia Clinica 2017IgG4-related disease is the term used to refer to a condition characterized by a lymphoplasmacytic infiltrate, fibrosis and an increased number of IgG4+ cells present in... (Review)
Review
IgG4-related disease is the term used to refer to a condition characterized by a lymphoplasmacytic infiltrate, fibrosis and an increased number of IgG4+ cells present in tissue, in most cases, with an elevated serum IgG4 level. This disease frequently affects the pancreas, salivary glands and lymph nodes, but can involve almost any tissue. Its etiology and the exact role of the different inflammatory cells in the damage to the target organ is still unclear. As yet, there is no international consensus about diagnostic criteria for the disease, but there are important advances in its treatment and in the quest to achieve remission. We include a review of the history, possible pathogenesis, clinical manifestations, diagnostic approach and available therapeutic approaches.
Topics: Autoimmune Diseases; Biomarkers; Global Health; Humans; Immunoglobulin G; Incidence; Prevalence
PubMed: 27329319
DOI: 10.1016/j.reuma.2016.05.009 -
Head and Neck Pathology Mar 2015IgG4 related disease of the head and neck region represents one of the more common manifestations of IgG4 related disease. Involvement of the submandibular and parotid... (Review)
Review
IgG4 related disease of the head and neck region represents one of the more common manifestations of IgG4 related disease. Involvement of the submandibular and parotid glands, the orbit and thyroid represent some of the more common sites involved by IgG4 related disease. Eosinophilic angiocentric fibrosis, Mikulicz disease and Riedel thyroiditis are also members of the family of IgG4 related disease. Clinically, the disease is characterized by tumefactive lesions, often multicentric, that show a swift response to immunosuppressive therapy. An elevated serum IgG4 represents the only validated blood based biomarker. However, elevated serum IgG4 is detected in only half the patients with this disease. Histology continues to represent the gold standard for the diagnosis of IgG4 related disease: storiform-type fibrosis and obliterative phlebitis constitute characteristic features of this disease. A definitive diagnosis of IgG4 related disease also requires the presence of elevated numbers of IgG4 positive plasma cells as well as an IgG4 to IgG ratio of greater than 40 %. In isolation, elevated numbers of IgG4 positive plasma cells represents a non-specific feature, detected in a variety of other inflammatory as well as neoplastic diseases. Attention to the clinical context, histological features, as well as an elevated IgG4 to IgG ratio is critical to avoiding overdiagnosis of IgG4 related disease.
Topics: Head; Humans; Immunoglobulin G; Neck
PubMed: 25804380
DOI: 10.1007/s12105-015-0620-6 -
Zeitschrift Fur Rheumatologie Apr 2022Immunoglobulin G (IgG) 4‑related diseases are fibrosing inflammatory systemic diseases that can affect almost every organ system. Typical disease manifestations...
Immunoglobulin G (IgG) 4‑related diseases are fibrosing inflammatory systemic diseases that can affect almost every organ system. Typical disease manifestations include autoimmune pancreatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory orbitopathy and involvement of the salivary and lacrimal glands. Organ involvements can occur either isolated or in combination with other disease manifestations. Before diagnosing IgG4-related diseases, malignancies and other inflammatory diseases have to be excluded. The diagnosis requires a combination of laboratory findings, histological and radiological results. Typically, IgG4-related diseases respond well to glucocorticosteroids. In cases of relapse or severe organ involvement a longer term immunosuppression is often required, whereas watch and wait can also be sufficient in milder cases.
Topics: Autoimmune Diseases; Fibrosis; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease
PubMed: 34851443
DOI: 10.1007/s00393-021-01130-z -
European Journal of Radiology Mar 2021Immunoglobulin G4-related disease (IgG4-RD) is a newly defined fibro-inflammatory multisystemic condition defined by a triad of diagnostic criteria based on clinical... (Review)
Review
PURPOSE
Immunoglobulin G4-related disease (IgG4-RD) is a newly defined fibro-inflammatory multisystemic condition defined by a triad of diagnostic criteria based on clinical presentation, biochemical and histopathological findings. Whereas some subsites of this disease have been well described in the literature so far (e.g. pancreas, kidneys, retroperitoneum, salivary glands), more recently identified anatomical sites of involvement in the head and neck are less well understood (e.g. nose, paranasal sinuses).
METHOD
This pictorial review details the imaging appearances of extracranial IgG4-RD in the Head & Neck. Multimodality imaging appearance and features are presented, with reference to the published literature to date.
RESULTS
Following a subsite-based approach, we present both the most common and the more rarely encountered imaging patterns of IgG4-RD in the extracranial head and neck, along with the relevant differential diagnoses to consider. Our institutional experience not only cements what is already known in the existing literature on this topic, but also reveals new imaging features of IgG4-RD, notably in the sinonasal tract.
CONCLUSIONS
This pictorial review of extracranial head & neck IgG4-RD will enable radiologists to recognise the features of this condition and propose it as a differential diagnosis to include alongside other probable entities. It establishes the place of the radiologist in the diagnosis and management of IgG4-RD.
Topics: Autoimmune Diseases; Head; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Neck
PubMed: 33524920
DOI: 10.1016/j.ejrad.2021.109560 -
Acta Otorhinolaryngologica Italica :... Apr 2017Autoimmune diseases of major salivary glands include Sjögren's syndrome and a complex of disorders classified as immunoglobulin G4-related diseases. These pathologies... (Review)
Review
Autoimmune diseases of major salivary glands include Sjögren's syndrome and a complex of disorders classified as immunoglobulin G4-related diseases. These pathologies are characterised by an autoimmune reaction mediated by T-helper lymphocytes that targets the ducts of exocrine glands in Sjögren's syndrome and glandular parenchyma in immunoglobulin G4-related diseases. Immunoglobulin G4-related diseases represent recently introduced multi-organ diseases that also involve the salivary glands. However, the morbid conditions once known as Mikulicz's disease and Kuttner's tumour were recently considered as two variants of immunoglobulin G4-related diseases affecting the major salivary glands ( immunoglobulin G4-related sialadenitis). This review briefly summarises the pathogenesis and clinical features of autoimmune diseases of the major salivary glands, focusing on the diagnostic and therapeutic role of sialendoscopy.
Topics: Autoimmune Diseases; Endoscopy; Humans; Immunoglobulin G; Sialadenitis; Sjogren's Syndrome
PubMed: 28516978
DOI: 10.14639/0392-100X-1605 -
Clinical and Experimental Immunology Aug 2015Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that derives its name from the characteristic finding of abundant IgG4(+) plasma cells in... (Review)
Review
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that derives its name from the characteristic finding of abundant IgG4(+) plasma cells in affected tissues, as well as the presence of elevated serum IgG4 concentrations in many patients. In contrast to fibrotic disorders, such as systemic sclerosis or idiopathic pulmonary fibrosis in which the tissues fibrosis has remained largely intractable to treatment, many IgG4-RD patients appear to have a condition in which the collagen deposition is reversible. The mechanisms underlying this peculiar feature remain unknown, but the remarkable efficacy of B cell depletion in these patients supports an important pathogenic role of B cell/T cell collaboration. In particular, aberrant T helper type 2 (Th2)/regulatory T cells sustained by putative autoreactive B cells have been proposed to drive collagen deposition through the production of profibrotic cytokines, but definitive demonstrations of this hypothesis are lacking. Indeed, a number of unsolved questions need to be addressed in order to fully understand the pathogenesis of IgG4-RD. These include the identification of an antigenic trigger(s), the implications (if any) of IgG4 antibodies for pathophysiology and the precise immunological mechanisms leading to fibrosis. Recent investigations have also raised the possibility that innate immunity might precede adaptive immunity, thus further complicating the pathological scenario. Here, we aim to review the most recent insights on the immunology of IgG4-RD, focusing on the relative contribution of innate and adaptive immune responses to the full pathological phenotype of this fibrotic condition. Clinical, histological and therapeutic features are also addressed.
Topics: Adaptive Immunity; B-Lymphocytes; Cell Communication; Collagen; Gene Expression; Granuloma, Plasma Cell; Humans; Immunity, Innate; Immunoglobulin G; Inflammation; Mikulicz' Disease; Retroperitoneal Fibrosis; T-Lymphocytes, Regulatory; Th2 Cells
PubMed: 25865251
DOI: 10.1111/cei.12641