Immunoglobulin G4 related chronic sclerosing sialadenitis

2015 ◽  
Vol 129 (3) ◽  
pp. 226-231 ◽  
Author(s):  
E L Culver ◽  
A Hunt ◽  
E Crewe ◽  
K A Shah ◽  
P Martinez-Devesa
Keyword(s):  
Literature Search ◽  
Systemic Disease ◽  
Clinical Suspicion ◽  
Cochrane Library ◽  
Disease Entity ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Chronic Sclerosing Sialadenitis ◽  
Search Terms ◽  
Salivary Gland Disease

AbstractBackground: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.

scholarly journals An Overlapping Case of IgG4-Related Disease and Systemic Lupus Erythematosus

2019 ◽  
Vol 7 ◽  
pp. 232470961986229 ◽  
Author(s):  
Srikanth Naramala ◽  
Sharmi Biswas ◽  
Sreedhar Adapa ◽  
Vijay Gayam ◽  
Venu Madhav Konala ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Disease ◽  
Lymph Node Biopsy ◽  
Systemic Lupus ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Double Stranded Dna ◽  
Serum Igg4

We are reporting a case of a 63-year-old Chinese female who presented to the rheumatology clinic with positive antinuclear antibody and unintentional weight loss along with lymphadenopathy. Further workup revealed eosinophilia, elevated anti–double stranded DNA, serum protein, and serum IgG4 (immunoglobulin G4). The patient was diagnosed with systemic lupus erythematosus. Due to the raised IgG4 level along with eosinophilia and diffuse lymphadenopathy, IgG4-related systemic disease was suspected. It was confirmed with IgG4 staining on lymph node biopsy. Our case is presenting the fact that systemic lupus erythematosus and IgG4-related disease can be present in the same patient with multiple overlapping features making accurate diagnosis challenging.

scholarly journals Thoracic Paravertebral Mass as an Infrequent Manifestation of IgG4-Related Disease

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Melissa Matzumura Kuan ◽  
Bernard Rubin ◽  
Alireza Meysami
Keyword(s):  
Submandibular Gland ◽  
Plasma Cells ◽  
Inflammatory Cells ◽  
Disease Recurrence ◽  
Tissue Mass ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Chronic Sclerosing Sialadenitis ◽  
Resistant Disease ◽  
Paravertebral Mass

Case. A 50-year-old African American male presented with abdominal pain and significant weight loss. On physical examination, he had parotid and submandibular gland enlargement associated with right eye proptosis. Computed tomography showed a thoracic paravertebral soft tissue mass, enlarged lymph nodes, and ascending aortic aneurysm. Laboratory results were remarkable for elevated total IgG and IgG4 subclass. The submandibular gland pathology revealed chronic sclerosing sialadenitis, with a large subset of inflammatory cells positively staining for IgG4. The histology of the paravertebral mass demonstrated fibrosclerosis with increased lymphocytic infiltrate, associated with increased IgG4 plasma cells. He was diagnosed with immunoglobulin G4-related disease (IgG4-RD). Steroid therapy initially yielded improvement; however, after steroids were stopped, there was disease recurrence. Prednisone was restarted, and the plan was to start him on rituximab. Interestingly, the patient’s brother also had IgG4-RD. Conclusion. IgG4-RD can present as a paravertebral mass which is usually responsive to steroids; however, recurrent and resistant disease can be seen for which steroid-sparing agents such as rituximab should be considered. In addition, to the best of our knowledge, this is the first reported case of IgG4-RD in two family members presenting as a paravertebral mass, highlighting an exciting area for more research in the future.

scholarly journals Epidemiology of Systemic Mycoses in the COVID-19 Pandemic

2021 ◽  
Vol 7 (7) ◽  
pp. 556
Author(s):  
María Guadalupe Frías-De-León ◽  
Rodolfo Pinto-Almazán ◽  
Rigoberto Hernández-Castro ◽  
Eduardo García-Salazar ◽  
Patricia Meza-Meneses ◽  
...  
Keyword(s):  
Diagnostic Tests ◽  
Literature Search ◽  
Demographic Data ◽  
Clinical Suspicion ◽  
Diagnostic Methods ◽  
Cochrane Library ◽  
Fungal Colonization ◽  
Systemic Mycosis ◽  
The Subject ◽  
Patient Prognosis

The physiopathologic characteristics of COVID-19 (high levels of inflammatory cytokines and T-cell reduction) promote fungal colonization and infection, which can go unnoticed because the symptoms in both diseases are very similar. The objective of this work was to study the current epidemiology of systemic mycosis in COVID-19 times. A literature search on the subject (January 2020–February 2021) was performed in PubMed, Embase, Cochrane Library, and LILACS without language restrictions. Demographic data, etiological agent, risk factors, diagnostic methods, antifungal treatment, and fatality rate were considered. Eighty nine publications were found on co-infection by COVID-19 and pneumocystosis, candidiasis, aspergillosis, mucormycosis, coccidioidomycosis, or histoplasmosis. In general, the co-infections occurred in males over the age of 40 with immunosuppression caused by various conditions. Several species were identified in candidiasis and aspergillosis co-infections. For diagnosis, diverse methods were used, from microbiological to molecular. Most patients received antifungals; however, the fatality rates were 11–100%. The latter may result because the clinical picture is usually attributed exclusively to SARS-CoV-2, preventing a clinical suspicion for mycosis. Diagnostic tests also have limitations beginning with sampling. Therefore, in the remainder of the pandemic, these diagnostic limitations must be overcome to achieve a better patient prognosis.

Head and neck immunoglobulin G4 related disease: systematic review

2018 ◽  
Vol 132 (12) ◽  
pp. 1046-1050 ◽  
Author(s):  
G Tirelli ◽  
N Gardenal ◽  
A Gatto ◽  
E Quatela ◽  
G C Del Piero
Keyword(s):  
Surgical Treatment ◽  
Medical Treatment ◽  
Head And Neck ◽  
Correct Diagnosis ◽  
Cochrane Library ◽  
Patient Specific ◽  
High Dose ◽  
Immunoglobulin G4 ◽  
Treatment Protocols ◽  
Related Disease

AbstractBackgroundImmunoglobulin G4 related disease is a recently described systemic syndrome. The head and neck region is the second most common site for presentation after the pancreas.MethodsPubMed and the Cochrane Library were searched from 1995 to July 2017 for all the studies on immunoglobulin G4 related disease diagnosed in the head and neck compartment. Patient-specific data were extracted and basic statistical analysis was performed.ResultsNinety-one patients were identified. Treatment was specified in 76 patients. Twenty patients received surgical treatment, eight of them in association with medical therapy. Fifty-six patients received medical treatment. The disease recurred in 25 per cent of patients treated with surgical treatment alone, in 3.6 per cent of patients treated with medical treatment alone and in 12.5 per cent of patients treated with both. All medical treatment protocols contained high-dose corticosteroids.ConclusionEarly and correct diagnosis can avoid unnecessary surgical treatment, and glucocorticoid therapy can improve the long-term prognosis.

scholarly journals Immunoglobulin G4-Related Disease Mimicking Asthma

2013 ◽  
Vol 20 (2) ◽  
pp. 87-89 ◽  
Author(s):  
Hiroshi Sekiguchi ◽  
Ryohei Horie ◽  
Timothy R Aksamit ◽  
Eunhee S Yi ◽  
Jay H Ryu
Keyword(s):  
Airway Inflammation ◽  
Submandibular Gland ◽  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Organ Manifestations

Immunoglobulin (Ig) G4-related disease (also known as ‘IgG4-related sclerosing disease’, ‘IgG4-related systemic disease’ or ‘hyper-IgG4-disease’) is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

scholarly journals Cross-sectional pictorial review of IgG4-related disease

2019 ◽  
Vol 92 (1103) ◽  
pp. 20190448 ◽  
Author(s):  
Darya Kurowecki ◽  
Michael N. Patlas ◽  
Ehsan A. Haider ◽  
Abdullah Alabousi
Keyword(s):  
Plasma Cells ◽  
Multidetector Ct ◽  
Current Knowledge ◽  
Systemic Disease ◽  
Autoimmune Disorder ◽  
Immunoglobulin G4 ◽  
Cross Sectional ◽  
Related Disease ◽  
Pictorial Review ◽  
Serum Igg4

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder characterized by the infiltration of one or more organs with IgG4-positive plasma cells resulting in inflammatory lesions and fibrosis. Although the pancreas is the most commonly affected organ, involvement of extrapancreatic organs is an increasingly recognized manifestation of the disease. Patients may be asymptomatic and serum IgG4 concentrations may be elevated or normal. Treatment consists of glucocorticosteroid treatment, with excellent response. A definitive diagnosis requires histopathology with imaging playing a key role in avoiding treatment delays. This pictorial review will focus on the most current knowledge regarding IgG4-RD including its common and less common manifestations and the roles of multidetector CT, MRI and ultrasound in the diagnosis and management of suspected IgG4-RD. Knowledge of the varied imaging findings of this multi systemic disease is essential for radiologists to avoid misdiagnosis and assist with timely and effective treatment.

scholarly journals Should civilian pre-hospital emergency care provision include tourniquets for the management of uncontrolled traumatic haemorrhage?

2015 ◽  
Vol 12 (4) ◽  
Author(s):  
Georgia Wright ◽  
Samantha Mcdonald ◽  
Gavin Smith
Keyword(s):  
Literature Search ◽  
Case Reports ◽  
Effective Means ◽  
Major Haemorrhage ◽  
Cochrane Library ◽  
Care Provision ◽  
Hospital Emergency ◽  
Search Terms ◽  
Academic Search ◽  
And Training

IntroductionTourniquets are used extensively in military settings, where they have proven to be an effective means of controlling major traumatic haemorrhage. This review aims to determine the value of tourniquets in the civilian pre-hospital setting.MethodA literature search was performed using the following electronic databases from inception to June 2014: Medline, Academic Search Premier and Cochrane Library. Additional searches were conducted in the Victoria University Library search engine. Key search terms included pre-hospital, tourniquet, military, complications, failure, ischemia, metabolic changes, history, and paramedics. Using the search terms outlined above, 236 articles were identified. With further examination 220 were excluded, leaving 16 for review (dated from 1974 - 2014). Of the 16 articles, 2 were determined to be relevant, published and peer reviewed case reports whilst the other 14 were peer reviewed and published studies.ResultsTourniquet use for controlling major haemorrhage is more accepted for military rather than civilian applications. Factors contributing to this include: lack of education and training, variation and incidence of specific mechanisms of injury, complications such as ischemia, decreased civilian scene times, and decreased civilian transport times to definitive care. Civilian research was inhibited by study numbers, with much of the data emanating from military research.ConclusionTo date, there is little evidence of tourniquet use in civilian environments. They are considered controversial due to complications and shortfalls. These factors contribute to user apprehension.

scholarly journals Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

2021 ◽  
Vol 12 ◽  
pp. 557
Author(s):  
Julie Mayeku ◽  
Jeremy Deisch ◽  
Miguel Angel Lopez-Gonzalez
Keyword(s):  
Cavernous Sinus ◽  
Plasma Cells ◽  
Surgical Intervention ◽  
Systemic Disease ◽  
Delayed Diagnosis ◽  
Unknown Etiology ◽  
Pathology Report ◽  
Partial Recovery ◽  
Immunoglobulin G4 ◽  
Related Disease

Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic disease of unknown etiology. It is characterized by tissue infiltration caused by IgG4 plasma cells and sclerosing inflammation of various body organs. At present, there are very few reported cases of IgG4-RD invasion of cavernous sinus and the orbit. Case Description: A 56-year-old female with a history of rheumatoid arthritis was presented with a gradual onset of right oculomotor, abducens, and trigeminal nerve deficits. Four weeks after the onset of symptoms, the patient developed gradual visual deficit. Following this, a trial of steroids was administered to the patient. However, the treatment did not work as expected and patient’s condition worsened. She progressed on to suffer complete visual loss in the right eye. Extensive work-up conducted on her turned out to be nondiagnostic. After this, the patient was referred to us for our evaluation. Neuroimaging revealed a right-sided cavernous sinus and orbital apex lesion. Given the lack of diagnosis and response to steroid treatment, we recommended surgical intervention and performed a modified pterional and pretemporal approach with extradural anterior clinoidectomy and transcavernous approach. We performed a lesion biopsy and cavernous sinus decompression, which helped in the partial recovery of visual function. The pathology report was consistent with IgG4-RD. Conclusion: IgG4-RD is a rare disease that occurs even less in combination with cavernous sinus and orbit invasion. The rarity of the disease and the diverse presentation of symptoms have sometimes caused delayed diagnosis and intervention. Patients who failed to respond to conservative management and patients in the fibrotic stage of the disease without other organ involvement may benefit from surgical intervention if amenable. Early suspicion, diagnosis, and intervention can facilitate better prognosis.

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