Ig G4

We present the case of a 56-year-old patient with fever of unknown origin associated with chest and lumbar pain. Multimodality imaging revealed diffuse peri-aortitis in the thoracic aorta without involvement of the aortic valve, contributing substantially to the diagnosis of Ig G4-associated aortitis. Immunosuppressive therapy was started. Follow-up at five months with cardiac magnetic resonance imaging showed a reduction in the inflammatory process in the thoracic aorta.

Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by elevated serum IgG4, IgG4+ cell infiltration, storiform fibrosis, and obliterative phlebitis. While IgG4-RD can affect various organs, gastrointestinal tract involvement is less common. Here, we report a 70-year-old female with IgG4-RD complicated with diffuse and chronic gastrointestinal inflammation which led to small intestinal perforation. She had been suffering from anorexia, abdominal pain, vomiting, and diarrhea, and hospitalized due to recurrent ileus...

RATIONALE: Immune-related adverse events following treatment with immune checkpoint inhibitors can affect almost every organ. Tislelizumab, a novel humanized Ig G4 programmed death receptor 1 inhibitor, was started for bladder cancer in 2019, but the adverse effects of this drug may not yet be known due to its short time on the market, and there are still some clinical safety concerns. There are few reports of adrenal insufficiency after tislelizumab treatment, which is easily missed, misdiagnosed and life-threatening...

We are writing in response to the paper published in the World Journal of Gastroenterology by Zhou et al . The authors identified higher serum immunoglobulin (Ig) G4 levels and age over 55 years as independent risk factors for disease relapse. Despite notable strengths, it is crucial to address potential biases. Firstly, the cohort study included 189 patients with autoimmune pancreatitis (AIP) type 1 (with higher IgG4 seropositivity and higher relapse) and 24 with type 2 (with lower IgG4 seropositivity and lower relapse)...

We report a case of immunoglobulin (ig)-g4-related thyroiditis associated with graves' disease. a 45-year-old man was diagnosed with graves' disease due to asymptomatic enlarged thyroid gland and high serum levels of thyrotropin receptor antibodies and thyroid hormones. surgical resection of the thyroid gland was performed because of further thyroid gland enlargement and severe fluctuations in the thyroid hormonal levels, despite medical therapy with a combination of an antithyroid drug and a thyroid hormone preparation...

BACKGROUND: Immunoglobulin G4 (Ig G4)-related disease is rare; however, it is a fibroinflammatory disease that has been studied a lot so far. Although the expression pattern varies depending on the organ affected, it usually manifests as organ hypertrophy and organ dysfunction. CASE PRESENTATION: A 46-year-old man was referred to our otorhinolaryngology department for left submandibular swelling and tenderness that occurred 2 weeks ago. He was treated with antibiotics (augmentin 625mg, per oral) for 2 weeks, but his symptoms did not improve, and his white blood cell (WBC) count was 10,500 /μL (normal 3,800-10,000 /μL)...

Autoimmune pancreatitis (AIP) is a rare disease. There are two distinct types of AIP: AIP type 1 (AIP-1), a pancreatic manifestation of a multi-organ disease linked to immunoglobulin (Ig)G4, and AIP type 2 (AIP-2), a pancreas-specific disease unrelated to IgG4. The usual course of treatment for AIP is oral corticosteroid medication. Rituximab has also been recommended for recurrent AIP-1 in order to initiate remission and provide ongoing treatment. Immunomodulators such as azathioprine are used to keep certain patients in remission...

BACKGROUND: The clinical, laboratory, and imaging characteristics of Hashimoto's thyroiditis are widely recognized. However, there is a dearth of information concerning the relationship between these aspects. The primary objective of this study was to investigate the correlation between sonographic features and immunologic parameters in individuals with Hashimoto's thyroiditis. METHODS: This cross-sectional study enrolled a cohort of 100 consecutive patients diagnosed with Hashimoto's thyroiditis...

Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease characterized by intrahepatic and extrahepatic bile duct strictures leading to cirrhosis. A subtype with elevated serum immunoglobulin (Ig) G4 levels has been recently identified. Elevated IgG4 titers can be present in 9%-15% of patients with PSC. Currently, liver transplantation is the only effective treatment of PSC, although multiple medical therapies are under evaluation. We report a case of a young adult with PSC and elevated IgG4 levels who had marked serum aminotransferase elevation; the patient had an incomplete response to steroids but achieved complete biochemical remission after initiation of oral vancomycin...

Immunoglobulin (Ig) G4 has a distinctive nature, and its involvement in autoimmune disorders is a subject of ongoing debate and uncertainty. A growing body of evidence indicates that IgG4 may play a pathogenic role in the development of systemic lupus erythematosus (SLE). The IgG4 autoantibodies have the capability to bind autoantigens in a competitive manner with other Ig classes, thereby forming immune complexes (ICs) that are noninflammatory in nature. This is due to the low affinity of IgG4 for both the Fc receptors and the C1 complement molecule, which results in a diminished inflammatory response in individuals with SLE...

Neurofibromatosis type 1 (NF1) is a disorder in which RAS is constitutively activated due to the loss of the Ras-GTPase-activating activity of neurofibromin. RAS must be prenylated (i.e., farnesylated or geranylgeranylated) to traffic and function properly. Previous studies showed that the anti-growth properties of farnesyl monophosphate prodrug farnesyltransferase inhibitors (FTIs) on human NF1 malignant peripheral nerve sheath tumor (MPNST) cells are potentiated by co-treatment with lovastatin. Unfortunately, such prodrug FTIs have poor aqueous solubility...

BCL6 translocation is one of the most common chromosomal translocations in cancer and results in its enhanced expression in germinal center B cells. It involves the fusion of BCL6 with any of its twenty-six Ig and non-Ig translocation partners associated with diffuse large B cell lymphoma (DLBCL). Despite being discovered long back, the mechanism of BCL6 fragility is largely unknown. Analysis of the translocation breakpoints in 5' UTR of BCL6 reveals the clustering of most of the breakpoints around a region termed Cluster II...

Autoimmune pancreatitis (AIP) is a complex, poorly understood disease gaining increasing attention. "Clinical Characteristics and Outcome of AIP Based on Serum IgG4 levels," investigated AIP with a focus on serum immunoglobulin (Ig) G4 levels. The 213 patients with AIP were classified according to serum IgG4 levels: Abnormal (elevated) and normal. Patients with higher IgG4 levels exhibited a more active immune system and increased relapse rates. Beyond IgG4, the IgA levels and age independently contributed to relapse risk, guiding risk assessment and tailored treatments for better outcomes...

t(8;14) translocation is the hallmark of Burkitt's lymphoma and results in c-MYC deregulation. During the translocation, the c-MYC gene on chromosome 8 gets juxtaposed to the Ig switch regions on chromosome 14. Although the promoter of c-MYC has been investigated for its mechanism of fragility, little is known about other c-MYC breakpoint regions. We have analysed the translocation break points at the exon 1/intron 1 of c-MYC locus from Burkitt's lymphoma patients. Results showed that breakpoint region, when present on a plasmid, could fold into an R-loop confirmation in a transcription-dependent manner...

BACKGROUND: Autoimmune pancreatitis (AIP) has been linked with elevated immunoglobulin (Ig) G4 levels. The characteristics and outcomes of AIP based on serum markers have not been fully evaluated. AIM: To compare clinical features, treatment efficacy, and outcome of AIP based on serum IgG4 levels and analyze predictors of relapse. METHODS: A total of 213 patients with AIP were consecutively reviewed in our hospital from 2006 to 2021. According to the serum IgG4 level, all patients were divided into two groups, the abnormal group ( n = 148) with a high level of IgG4 [> 2 × upper limit of normal (ULN)] and the normal group ( n = 65)...

BACKGROUND: Multinucleated giant cells (MGCs) in bladder carcinomas are poorly studied. AIM: To describe the function, morphogenesis, and origin of mononuclear and MGCs in urothelial carcinoma (UC) of the bladder in Bulgarian and French patients. METHODS: Urothelial bladder carcinomas ( n = 104) from 2016-2020 were analyzed retrospectively using immunohistochemical (IHC) and histochemical stain examination. Giant cells in the bladder stroma were found in 35...

Immunoglobulin (Ig) genes carry the unique ability to be reshaped in peripheral B lymphocytes after these cells encounter a specific antigen. B cells can then further improve their affinity, acquire new functions as memory cells and eventually end up as antibody-secreting cells. Ig class switching is an important change that occurs in this context, thanks to local DNA lesions initiated by the enzyme activation-induced deaminase (AID). Several cis-acting elements of the Ig heavy (IgH) chain locus make it accessible to the AID-mediated lesions that promote class switch recombination (CSR)...

Interchain disulfide bonds in monoclonal antibodies may be reduced during large-scale mAb production using Chinese hamster ovary (CHO) cells. This reaction lowers the mAb product yield and purity; however, it may be prevented by screening cell lines that are unsusceptible to reduction and using them in mAb production. Antibody reduction susceptibility may be cell line-dependent. To the best of our knowledge, however, an efficient method of screening reduction-unsusceptible CHO cell lines has not been previously reported...

Muscle-specific kinase (MuSK) Myasthenia Gravis (MG) represents a prototypical antibody-mediated disease characterized by predominantly focal muscle weakness (neck, facial, and bulbar muscles) and fatigability. The pathogenic antibodies mostly belong to the immunoglobulin subclass (Ig)G4, a feature which attributes them their specific properties and pathogenic profile. On the other hand, acetylcholine receptor (AChR) MG, the most prevalent form of MG, is characterized by immunoglobulin (Ig)G1 and IgG3 antibodies to the AChR...

Immunoglobulin (Ig) G4 accounts for 4-6% of the total IgG in a healthy human. Several evidence-based studies have suggested that the level of IgG4 is significantly elevated in autoimmune diseases, including rheumatoid arthritis (RA). The clinical significance of IgG4 in RA with regard to disease activity, severity, and treatment response remains elusive. We consecutively recruited 174 patients with RA from our rheumatology clinic. All subjects were assessed for their disease activity based on DAS28, radiographic joint damage based on the Modified Sharp Score (MSS), the functional capacity based on the Health Assessment Questionnaire -Disability Index (HAQ-DI), and treatment responsiveness using the European League Against Rheumatism (EULAR) response criteria...