scholarly journals Autoimmune Diseases of Digestive Organs—A Multidisciplinary Challenge: A Focus on Hepatopancreatobiliary Manifestation

2021 ◽  
Vol 10 (24) ◽  
pp. 5796
Author(s):  
Lumir Kunovsky ◽  
Petr Dite ◽  
Petr Jabandziev ◽  
Zdenek Kala ◽  
Jitka Vaculova ◽  
...  
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Human Leukocyte ◽  
Typical Feature ◽  
The Body ◽  
Primary Biliary Cholangitis ◽  
Immunoglobulin G4 ◽  
Leukocyte Antigen ◽  
Appropriate Treatment ◽  
Mononuclear Infiltrate

It is well known that some pathological conditions, especially of autoimmune etiology, are associated with the HLA (human leukocyte antigen) phenotype. Among these diseases, we include celiac disease, inflammatory bowel disease, autoimmune enteropathy, autoimmune hepatitis, primary sclerosing cholangitis and primary biliary cholangitis. Immunoglobulin G4-related diseases (IgG4-related diseases) constitute a second group of autoimmune gastrointestinal, hepatobiliary and pancreatic illnesses. IgG4-related diseases are systemic and rare autoimmune illnesses. They often are connected with chronic inflammation and fibrotic reaction that can occur in any organ of the body. The most typical feature of these diseases is a mononuclear infiltrate with IgG4-positive plasma cells and self-sustaining inflammatory response. In this review, we focus especially upon the hepatopancreatobiliary system, autoimmune pancreatitis and IgG4-related sclerosing cholangitis. The cooperation of the gastroenterologist, radiologist, surgeon and histopathologist is crucial for establishing correct diagnoses and appropriate treatment, especially in IgG4 hepatopancreatobiliary diseases.

scholarly journals Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

scholarly journals Manifestations of immunoglobulin G4 related disease in otolaryngology: case reports and review of the literature

2014 ◽  
Vol 128 (S2) ◽  
pp. S10-S15 ◽  
Author(s):  
W K Wong ◽  
R Campbell ◽  
R Douglas
Keyword(s):  
B Lymphocyte ◽  
Plasma Cells ◽  
Case Reports ◽  
Correct Diagnosis ◽  
Infratemporal Fossa ◽  
The Body ◽  
Infraorbital Nerve ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Storiform Fibrosis

AbstractBackground:Immunoglobulin G4 related disease is an inflammatory condition characterised by the presence of fibrotic lesions infiltrated by immunoglobulin G4 positive plasma cells. It can arise from almost any region of the body and it is being increasingly recognised in the head and neck. Regardless of the site of involvement, the histopathological resemblance is remarkable. Dense lymphoplasmacytic infiltration, overabundance of immunoglobulin G4 bearing plasma cells and presence of storiform fibrosis are typical findings.Case reports:This paper presents two cases of immunoglobulin G4 related disease in which there was involvement of the orbit, the infraorbital nerve and the infratemporal fossa. Diagnosis was established in both cases by biopsying radiologically abnormal tissue in the infratemporal fossa.Conclusion:An awareness of this condition is required to establish the diagnosis and initiate appropriate therapy. Glucocorticoids are the mainstay of initial treatment. The effectiveness of B-lymphocyte depletion with rituximab has also been reported. Correct diagnosis may spare patients from unnecessarily radical surgery.

scholarly journals Association of Human Leukocyte Antigens Class II Variants with Susceptibility to Hidradenitis Suppurativa in a Caucasian Spanish Population

2020 ◽  
Vol 9 (10) ◽  
pp. 3095
Author(s):  
J. Gonzalo Ocejo-Vinyals ◽  
Miguel A. Gonzalez-Gay ◽  
Marcelo A. Fernández-Viña ◽  
Juan Cantos-Mansilla ◽  
Iosune Vilanova ◽  
...  
Keyword(s):  
Hidradenitis Suppurativa ◽  
Human Leukocyte ◽  
Class Ii ◽  
The Body ◽  
Northern Spain ◽  
Allele Distribution ◽  
Leukocyte Antigen ◽  
Statistical Correction ◽  
Antigen System ◽  
Dqb1 Allele

Hidradenitis suppurativa (HS) is a chronic inflammatory cutaneous disease of the hair follicle typically presenting recurrent, painful, and inflamed lesions on the inverse areas of the body. Although its pathogenesis remains unknown, the immune system appears to play a potential role. To date, two previous studies have not found any association between the Human Leukocyte Antigen system (HLA) and HS. In this study we analyzed the HLA-A, -B, -C; and DRB1, -DQA1, and –DQB1 allele distribution in 106 HS patients and 262 healthy controls from a Caucasian population in Cantabria (northern Spain). HLA-A*29 and B*50 were significantly more common in HS patients and A*30 and B*37 in controls, but these associations disappeared after statistical correction. DRB1*07, DQA1*02, and DQB1*02 were significantly more common in controls (p 0.026, p 0.0012, and p 0.0005, respectively) and the HLA allele DQB1*03:01 was significantly more common in HS patients (p 0.00007) after the Bonferroni correction. The DRB1*07~DQA1*02~DQB1*02 haplotype was significantly more common in controls (p < 0.0005). This is the first study showing an association between HLA-class II and HS. Our results suggest that HLA-II alleles (DRB1*07, DQA1*02, DQB1*02, and DQB1*03:01) and the DRB1*07~DQA1*02~DQB1*02 haplotype could influence resistance or susceptibility to HS.

scholarly journals The pathogenesis of ankylosing spondylitis

2008 ◽  
Vol 24 (1) ◽  
pp. E3 ◽  
Author(s):  
Mohammed F. Shamji ◽  
Mohammed Bafaquh ◽  
Eve Tsai
Keyword(s):  
Ankylosing Spondylitis ◽  
Molecular Mimicry ◽  
Human Leukocyte ◽  
Axial Skeleton ◽  
Chronic Inflammatory Disease ◽  
The Body ◽  
Hla B27 ◽  
Leukocyte Antigen ◽  
Molecular Events ◽  
Microbial Tolerance

✓ Ankylosing spondylitis (AS) is a chronic inflammatory disease that can cause significant functional complications by affecting the sacroiliac joints and axial skeleton. Despite a longstanding knowledge about the familial associations of this disease, particularly among patients positive for human leukocyte antigen (HLA)–B27, the fundamental pathogenetic mechanism by which this disease arises in genetically susceptible individuals remains ill defined. Furthermore, the molecular predilection for characteristic articular site involvement remains under ongoing investigation. Current theories about the HLA-B27 association range from the presentation of novel arthritogenic peptides, to abnormal autoimmune stimulation, to anomalous microbial tolerance. The immune effectors of this damage include CD4+, CD8+, and natural killer cells, with marked heterogeneity at different sites. Biomechanical stresses may trigger this disease by exposing the body to previously immune-sequestered autoantigens or by providing a route for bacterial seeding. Environmental triggers such as infection have not been definitively established but may represent a primary pathogenic step in a molecular-mimicry process. In this article, the authors review the current literature on the origin and pathophysiology of AS, focusing on genetic and molecular associations, consequent pathomechanisms, and associated triggers. An improved understanding of the sequence of molecular events that predispose and initiate the onset of this disease will allow for more specific and targeted therapy and better avoidance of the significant side effects of systemic immunomodulation.

scholarly journals Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Pragya Shrestha ◽  
Brian Le ◽  
Brent Wagner ◽  
William Pompella ◽  
Paras Karmacharya
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Clinical Manifestations ◽  
Disease Process ◽  
High Serum ◽  
Diagnostic Challenge ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Serum Igg4

IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.

scholarly journals CD86+ or HLA-G+ can be transferred via trogocytosis from myeloma cells to T cells and are associated with poor prognosis

Blood ◽  
2012 ◽  
Vol 120 (10) ◽  
pp. 2055-2063 ◽  
Author(s):  
Ross Brown ◽  
Karieshma Kabani ◽  
James Favaloro ◽  
Shihong Yang ◽  
P. Joy Ho ◽  
...  
Keyword(s):  
T Cells ◽  
T Cell ◽  
Poor Prognosis ◽  
Plasma Cells ◽  
Side Population ◽  
Immune Surveillance ◽  
Human Leukocyte ◽  
Cell Receptor ◽  
Lymphocytic Leukemia ◽  
Leukocyte Antigen

Abstract The transfer of membrane proteins between cells during contact, known as trogocytosis, can create novel cells with a unique phenotype and altered function. We demonstrate that trogocytosis is more common in multiple myeloma (MM) than chronic lymphocytic leukemia and Waldenstrom macroglobulinaemia; that T cells are more probable to be recipients than B or natural killer cells; that trogocytosis occurs independently of either the T-cell receptor or HLA compatibility; and that after trogocytosis, T cells with acquired antigens can become novel regulators of T-cell proliferation. We screened 168 patients with MM and found that CD86 and human leukocyte antigen G (HLA-G) were antigens commonly acquired by T cells from malignant plasma cells. CD3+CD86acq+ and CD3+ HLA-Gacq+ cells were more prevalent in bone marrow than peripheral blood samples. The presence of either CD86 or HLA-G on malignant plasma cells was associated with a poor prognosis. CD38++ side population cells expressed HLA-G, suggesting that these putative myeloma stem cells could generate immune tolerance. HLA-G+ T cells had a regulatory potency similar to natural Tregs, thus providing another novel mechanism for MM to avoid effective immune surveillance.

scholarly journals Expression of peripheral blood monocyte human leukocyte antigen DR in patients with cardiac arrest and its clinical significance

2018 ◽  
Vol 27 (1) ◽  
pp. 3-7
Author(s):  
Junli Zhang ◽  
Yanjuan Wang ◽  
Wei Gu
Keyword(s):  
Cardiac Arrest ◽  
Human Leukocyte Antigen ◽  
Immune Function ◽  
Clinical Significance ◽  
Human Leukocyte ◽  
The Body ◽  
Apache Ii ◽  
Survival Group ◽  
Leukocyte Antigen ◽  
Death Group

Background: In the early period of spontaneous circulation (ROSC), the body may show severe immunosuppression and excessive activation of inflammatory response, This is very similar to sepsis in many ways. Objective: The aim of this study is to observe changes of the early expression of monocyte human leukocyte antigen DR in patients with cardiac arrest, so as to explore the clinical significance of the related immune assessment and prognosis prediction. Methods: A total of 43 patients with cardiac arrest who have been treated in the emergency department of Beijing Chaoyang Hospital from January 2015 to February 2018 are selected. By taking the survival rate on the 28th day of hospitalization as the end of observation, the patients are divided into the survival group and the death group. Changes of APACHE-II scores and monocyte human leukocyte antigen DR levels on the first, second, and third day after admission are analyzed. Results: On the first, second, and third day after onset, cardiac arrest patients show significantly decreased levels of monocyte human leukocyte antigen DR which are obviously lower in the death group than in the survival group. In addition, human leukocyte antigen DR levels were significantly negatively correlated with APACHE-II scores. Conclusion: The expression of monocyte human leukocyte antigen DR is proven to be an ideal indicator to evaluate the immune function and prognosis of cardiac arrest patients. A constantly low expression of human leukocyte antigen DR indicates impaired immune function and increased mortality of patients.

scholarly journals Bioinformatic HLA Studies in the Context of SARS-CoV-2 Pandemic and Review on Association of HLA Alleles with Preexisting Medical Conditions

2021 ◽  
Vol 2021 ◽  
pp. 1-12
Author(s):  
Mina Mobini Kesheh ◽  
Sara Shavandi ◽  
Parastoo Hosseini ◽  
Rezvan Kakavand-Ghalehnoei ◽  
Hossein Keyvani
Keyword(s):  
Mortality Rate ◽  
Healthcare Workers ◽  
Human Leukocyte ◽  
The Body ◽  
Medical Conditions ◽  
Hla Alleles ◽  
Leukocyte Antigen ◽  
Polymorphic Genes ◽  
Global Concern ◽  
Severity Of The Disease

After the announcement of a new coronavirus in China in December 2019, which was then called SARS-CoV-2, this virus changed to a global concern and it was then declared as a pandemic by WHO. Human leukocyte antigen (HLA) alleles, which are one of the most polymorphic genes, play a pivotal role in both resistance and vulnerability of the body against viruses and other infections as well as chronic diseases. The association between HLA alleles and preexisting medical conditions such as cardiovascular diseases and diabetes mellitus is reported in various studies. In this review, we focused on the bioinformatic HLA studies to summarize the HLA alleles which responded to SARS-CoV-2 peptides and have been used to design vaccines. We also reviewed HLA alleles that are associated with comorbidities and might be related to the high mortality rate among COVID-19 patients. Since both genes and patients’ medical conditions play a key role in both severity of the disease and the mortality rate in COVID-19 patients, a better understanding of the connection between HLA alleles and SARS-CoV-2 can provide a wider perspective on the behavior of the virus. Such understanding can help scientists, especially in terms of protecting healthcare workers and designing effective vaccines.

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