scholarly journals Immunoglobulin G4-related Disease: A New Systemic Disease Emerging in Japan

JMA Journal ◽  
2022 ◽  
Vol 5 (1) ◽  
pp. 23-35
Keyword(s):  
Systemic Disease ◽  
Immunoglobulin G4 ◽  
Related Disease

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 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 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.

FDG PET/CT in Isolated Mediastinal Immunoglobulin G4–Related Disease

2020 ◽  
Vol 45 (4) ◽  
pp. e195-e197
Author(s):  
Vankadari Kousik ◽  
Rajender Kumar ◽  
Bhagwant Rai Mittal ◽  
Divya Aggarwal ◽  
Ashutosh Nath Aggarwal
Keyword(s):  
Fdg Pet ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Pet Ct ◽  
Fdg Pet Ct

scholarly journals Evaluate the Differences in CT Features and Serum IgG4 Levels between Lymphoma and Immunoglobulin G4-Related Disease of the Orbit

2020 ◽  
Vol 9 (8) ◽  
pp. 2425
Author(s):  
Wei-Hsin Yuan ◽  
Anna Fen-Yau Li ◽  
Shu-Yi Yu ◽  
Ying-Yuan Chen ◽  
Chia-Hung Wu ◽  
...  
Keyword(s):  
Elevated Serum ◽  
Hounsfield Unit ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Serum Igg4 ◽  
Orbital Lymphoma ◽  
Serum Igg Levels ◽  
Igg Level ◽  
Ct Features ◽  
Higher Sensitivity

Background: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)—characterized as tumors mimicking malignant orbital lymphoma (OL)—responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. Methods: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. Results: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions—involving both lacrimal glands on CT scans—higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674–1.000, p = 0.005] vs. 0.766 [95% CI: 0.615–0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518–0.997] vs. 0.75 [95% CI: 0.509–0.913]), higher specificity (0.813 [95% CI: 0.544–0.960] vs. 0.778 [95% CI: 0.578–0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). Conclusions: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.

scholarly journals A Case of IgG4-Related Kidney Disease Developing While on Steroid Treatment for Autoimmune IgG4 Pancreatitis

2021 ◽  
Vol 9 ◽  
pp. 232470962110265
Author(s):  
Jonathan Vincent M. Reyes ◽  
Dawn Maldonado ◽  
Aaron S. Stern ◽  
Maritza Brown
Keyword(s):  
Kidney Disease ◽  
Inflammatory Process ◽  
Systemic Disease ◽  
Multiple Organ ◽  
Steroid Treatment ◽  
Autoimmune Process ◽  
Immunoglobulin G4 ◽  
Organ Systems ◽  
The Relationship ◽  
Steroid Sparing

IgG4 (immunoglobulin G4)-related systemic disease is an autoimmune process affecting multiple organ systems. This inflammatory process can present as but not limited to pancreatitis, cholangitis, or unspecified kidney disease. In this case, our patient developed IgG4-related kidney disease while already on a prolonged steroid course for IgG4-related pancreatitis. The patient ultimately had renal recovery after starting a higher dose of prednisone, but also developed steroid-related complications. This case further highlights the relationship between IgG4 diseases now termed IgG4-related systemic disease. This case brings to light the need for further investigative research into ideal steroid dosing, as well as steroid-sparing agents for IgG4-related systemic disease.

Immunoglobulin G4–related disease of the paratestis: Can we avoid radical treatment?

2020 ◽  
pp. 205141582098277
Author(s):  
Thomas Thompson ◽  
Thomas Brophy ◽  
Mohsin Uddin ◽  
James Bolton ◽  
Richard Napier-Hemy
Keyword(s):  
Immunoglobulin G4 ◽  
Related Disease ◽  
Radical Treatment
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