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Common Variable Immune disease

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https://www.readbyqxmd.com/read/29019451/autoimmunity-in-a-cohort-of-471-patients-with-primary-antibody-deficiencies
#1
Gholamreza Azizi, Marzieh Tavakol, Hosein Rafiemanesh, Fatemeh Kiaee, Reza Yazdani, Amin Heydari, Kosar Abouhamzeh, Pardis Anvari, Sara Mohammadikhajehdehi, Laleh Sharifia, Yasser Bagheri, Hamed Mohammadi, Hassan Abolhassani, Asghar Aghamohammadi
OBJECTIVES: The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD). METHODS: A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients' demographic information, clinical records and laboratory data were collected to investigate autoimmune complications. RESULTS: Autoimmune disorders as the first presentation of immunodeficiency were recorded in 11 patients (2...
November 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28983810/dysregulation-of-innate-lymphoid-cells-in-common-variable-immunodeficiency
#2
REVIEW
Paul J Maglione, Montserrat Cols, Charlotte Cunningham-Rundles
Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immune deficiency. With widespread use of immunoglobulin replacement therapy, non-infectious complications, such as autoimmunity, chronic intestinal inflammation, and lung disease, have replaced infections as the major cause of morbidity and mortality in this immune deficiency. The pathogenic mechanisms that underlie the development of these complications in CVID are not known; however, there have been numerous associated laboratory findings...
October 5, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28983794/somatic-hypermutation-defects-in-common-variable-immune-deficiency
#3
REVIEW
María Belén Almejun, Mercedes Borge
Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by impaired antibody production and recurrent infections. In the last 20 years, several groups have reported that B cells from CVID patients have an impaired somatic hypermutation (SHM). The reported frequency of this defect among CVID patient cohorts is highly variable and so is the methodology used to evaluate this process. Interestingly, the low level of SHM on B cells from CVID patients has been correlated with the presence of infectious and non-infectious complications...
October 5, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28983790/influenza-vaccination-in-patients-with-common-variable-immunodeficiency-cvid
#4
REVIEW
Jan F Mieves, Kirsten Wittke, Helma Freitag, Hans-Dieter Volk, Carmen Scheibenbogen, Leif G Hanitsch
PURPOSE OF REVIEW: Vaccination against influenza in patients with primary antibody deficiency is recommended. Common variable immunodeficiency (CVID) is the most frequent and clinically relevant antibody deficiency disease and is by definition characterized by an impaired vaccination response. The purpose of this review is to present the current knowledge of humoral and cellular vaccine response to influenza in CVID patients. RECENT FINDINGS: Studies conducted in CVID patients demonstrated an impaired humoral response upon influenza vaccination...
October 5, 2017: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/28969386/genetic-variants-influencing-elevated-myeloperoxidase-levels-increase-risk-of-stroke
#5
Chia-Ling Phuah, Tushar Dave, Rainer Malik, Miriam R Raffeld, Alison M Ayres, Joshua N Goldstein, Anand Viswanathan, Steven M Greenberg, Jeremiasz M Jagiella, Björn M Hansen, Bo Norrving, Jordi Jimenez-Conde, Jaume Roquer, Alexander Pichler, Christian Enzinger, Joan Montaner, Israel Fernandez-Cadenas, Arne Lindgren, Agnieszka Slowik, Reinhold Schmidt, Alessandro Biffi, Natalia Rost, Carl D Langefeld, Hugh S Markus, Braxton D Mitchell, Brad B Worrall, Steven J Kittner, Daniel Woo, Martin Dichgans, Jonathan Rosand, Christopher D Anderson
Primary intracerebral haemorrhage and lacunar ischaemic stroke are acute manifestations of progressive cerebral microvascular disease. Current paradigms suggest atherosclerosis is a chronic, dynamic, inflammatory condition precipitated in response to endothelial injury from various environmental challenges. Myeloperoxidase plays a central role in initiation and progression of vascular inflammation, but prior studies linking myeloperoxidase with stroke risk have been inconclusive. We hypothesized that genetic determinants of myeloperoxidase levels influence the development of vascular instability, leading to increased primary intracerebral haemorrhage and lacunar stroke risk...
October 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28967977/nail-involvement-in-alopecia-areata-a-questionnaire-based-survey-of-impact-on-quality-of-life-and-review-of-the-literature
#6
Yvonne B M Roest, Henrià T van Middendorp, Andrea W M Evers, Peter C M van de Kerkhof, Marcel C Pasch
Alopecia areata (AA) is an immune-mediated disease causing temporary or permanent hair loss. Up to 46% of patients with AA also have nail involvement. The aim of this study was to determine the presence, types, and clinical implications of nail changes in patients with AA. This questionnaire-based survey evaluated 256 patients with AA. General demographic variables, specific nail changes, nail-related quality of life (QoL), and treatment history and need were evaluated. Prevalence of nail involvement in AA was 64...
October 2, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28945243/germline-bias-dictates-cross-serotype-reactivity-in-a-common-dengue-virus-specific-cd8-t-cell-response
#7
Abigail Culshaw, Kristin Ladell, Stephanie Gras, James E McLaren, Kelly L Miners, Carine Farenc, Heleen van den Heuvel, Emma Gostick, Wanwisa Dejnirattisai, Apirath Wangteeraprasert, Thaneeya Duangchinda, Pojchong Chotiyarnwong, Wannee Limpitikul, Sirijitt Vasanawathana, Prida Malasit, Tao Dong, Jamie Rossjohn, Juthathip Mongkolsapaya, David A Price, Gavin R Screaton
Adaptive immune responses protect against infection with dengue virus (DENV), yet cross-reactivity with distinct serotypes can precipitate life-threatening clinical disease. We found that clonotypes expressing the T cell antigen receptor (TCR) β-chain variable region 11 (TRBV11-2) were 'preferentially' activated and mobilized within immunodominant human-leukocyte-antigen-(HLA)-A*11:01-restricted CD8(+) T cell populations specific for variants of the nonstructural protein epitope NS3133 that characterize the serotypes DENV1, DENV3 and DENV4...
September 25, 2017: Nature Immunology
https://www.readbyqxmd.com/read/28936778/preferential-reduction-of-circulating-innate-lymphoid-cells-type-2-in-patients-with-common-variable-immunodeficiency-with-secondary-complications-is-part-of-a-broader-immune-dysregulation
#8
David Friedmann, Baerbel Keller, Ina Harder, Jonas Schupp, Yakup Tanriver, Susanne Unger, Klaus Warnatz
PURPOSE: Over a third of patients with common variable immunodeficiency (CVID) suffer from secondary complications like inflammatory organ disease, autoimmune manifestations, or lymphoproliferation contributing to increased morbidity and mortality in affected patients. Innate lymphoid cells (ILCs) have emerging roles in setting the milieu for physiological, but also pathological, immune responses and inflammation. We therefore sought to correlate the recently identified disturbed homeostasis of ILCs with alterations of the adaptive immune system in complex CVID patients (CVIDc)...
September 21, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#9
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28895035/splenectomy-vs-rituximab-as-a-second-line-therapy-in-immune-thrombocytopenic-purpura-a-single-center-experience
#10
Ahmed S Al Askar, Naila A Shaheen, Mohsen Al Zahrani, Mohammed G Al Otaibi, Bader S Al Qahtani, Faris Ahmed, Mohand Al Zughaibi, Ismat Kamran, May Anne Mendoza, Altaf Khan
Immune thrombocytopenic purpura (ITP) is a common hematological disease treated primarily by corticosteroids. The aim of the present study was to compare response rate between patients, underwent splenectomy vs. rituximab as second-line therapy. Adult patients diagnosed with ITP who did not respond to corticosteroids or relapsed during the period 1990-2014 were included in a quasi-experimental study. Categorical variables were compared using Fisher exact test. Response to treatment was compared using logistic regression...
September 11, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28878085/hcmv-particles-treated-with-specific-antibodies-induce-intrinsic-and-adaptive-but-not-innate-immune-responses
#11
Zeguang Wu, Ruifang Qin, Li Wang, Matteo Bosso, Myriam Scherer, Thomas Stamminger, Dominik Hotter, Thomas Mertens, Giada Frascaroli
Human cytomegalovirus (HCMV) persistently infects 40% to 100% of the human population worldwide. Experimental and clinical evidence indicates that humoral immunity to HCMV plays an important role in restricting virus dissemination and protecting the infected host from disease. Specific immunoglobulin preparations from pooled plasma of adults selected for high titers of HCMV antibodies, have been used for prevention of CMV disease in transplant recipients and pregnant women. Even though incubation of HCMV particles with these preparations leads to neutralization of the viral infectivity, it is still unclear whether the antibody-treated HCMV particles (here defined as HCMV-Ab) enter into the cells and modulate anti-viral immune responses...
September 6, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28877998/respiratory-infections-and-the-risk-of-celiac-disease
#12
Renata Auricchio, Donatella Cielo, Renato de Falco, Martina Galatola, Valentina Bruno, Basilio Malamisura, Maria Giovanna Limongelli, Riccardo Troncone, Luigi Greco
BACKGROUND AND OBJECTIVES: The increasing incidence of celiac disease (CD) suggests that common infections before the onset of autoimmune diseases could be an important factor in switching the immune response. We aimed to explore the relationship between early clinical events and the development of CD in genetically predisposed infants. METHODS: In this study, 373 newborns from families with at least 1 relative with CD were recruited, and human leukocyte antigen DQ2- or DQ8-positive infants were followed up with clinical and serological evaluations...
October 2017: Pediatrics
https://www.readbyqxmd.com/read/28869867/atopic-dermatitis-and-psoriasis-two-different-immune-diseases-or-one-spectrum
#13
REVIEW
Emma Guttman-Yassky, James G Krueger
Psoriasis and atopic dermatitis (AD) are common T-cell mediated inflammatory diseases of the skin that can be treated by specific cytokine antagonists or more broad immunosuppressive drugs. The diseases are similar in that epidermal keratinocytes respond to T-cell derived cytokines by altering growth and differentiation responses, accounting for major parts of the overall disease phenotype. When studied across European-American populations, psoriasis and AD display differing T-cell polarity and different arrays of cytokines...
September 1, 2017: Current Opinion in Immunology
https://www.readbyqxmd.com/read/28827716/a-multi-parametric-analysis-of-trypanosoma-cruzi-infection-common-pathophysiologic-patterns-beyond-extreme-heterogeneity-of-host-responses
#14
Julien Santi-Rocca, Fernando Fernandez-Cortes, Carlos Chillón-Marinas, María-Luisa González-Rubio, David Martin, Núria Gironès, Manuel Fresno
The extreme genetic diversity of the protozoan Trypanosoma cruzi has been proposed to be associated with the clinical outcomes of the disease it provokes: Chagas disease (CD). To address this question, we analysed the similarities and differences in the CD pathophysiogenesis caused by different parasite strains. Using syngeneic mice infected acutely or chronically with 6 distant parasite strains, we integrated simultaneously 66 parameters: parasite tropism (7 parameters), organ and immune responses (local and systemic; 57 parameters), and clinical presentations of CD (2 parameters)...
August 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28812000/diagnostic-imaging-features-of-necrotizing-enterocolitis-a-narrative-review
#15
REVIEW
Francesco Esposito, Rosanna Mamone, Marco Di Serafino, Carmela Mercogliano, Valerio Vitale, Gianfranco Vallone, Patrizia Oresta
Necrotizing enterocolitis (NEC) is an inflammatory process, characterized by intestinal necrosis of variable extension, leading to perforation, generalized peritonitis and death. The classical pathogenetic theory focuses on mucosal damage related to a stress induced intestinal ischemia leading to mucosal injury and bacterial colonization of the wall. A more recent hypothesis emphasizes the role of immaturity of gastrointestinal and immune system, particularly of the premature, responsible of bowel wall vulnerability and suffering...
June 2017: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/28810029/celiac-disease-and-nonceliac-gluten-sensitivity-a-review
#16
REVIEW
Maureen M Leonard, Anna Sapone, Carlo Catassi, Alessio Fasano
Importance: The prevalence of gluten-related disorders is rising, and increasing numbers of individuals are empirically trying a gluten-free diet for a variety of signs and symptoms. This review aims to present current evidence regarding screening, diagnosis, and treatment for celiac disease and nonceliac gluten sensitivity. Observations: Celiac disease is a gluten-induced immune-mediated enteropathy characterized by a specific genetic genotype (HLA-DQ2 and HLA-DQ8 genes) and autoantibodies (antitissue transglutaminase and antiendomysial)...
August 15, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/28799803/nutrition-inflammation-and-liver-spleen-axis
#17
Luigi Barrea, Carolina Di Somma, Giovanna Muscogiuri, Giovanni Tarantino, Gian Carlo Tenore, Francesco Orio, Annamaria Colao, Silvia Savastano
Chronic low-grade systemic inflammation represents a mechanism common to many diseases linked to atherosclerosis-related pathways. There is a growing body of evidence indicating that the combination of food quantity and quality along with genetic susceptibility are able to induce the aberrant activation of innate immune signalling, which initially contributes to chronic low-grade inflammation. Liver represents the central player to inflammatory response. Dietary/metabolic factors contribute to the pathogenesis of Non-alcoholic Fatty Liver Disease (NAFLD), the main causes of liver disease in the Western world...
August 11, 2017: Critical Reviews in Food Science and Nutrition
https://www.readbyqxmd.com/read/28791687/skin-signs-of-primary-immunodeficiencies-how-to-find-the-genes-to-check
#18
REVIEW
M Ettinger, J Schreml, K Wirsching, M Berneburg, S Schreml
Primary immunodeficiencies (PIDs) are a heterogeneous group of rare diseases that result from defects in immune system development and/or function. The clinical manifestations of PIDs are highly variable, but most disorders involve at least an increased susceptibility to infection. Furthermore, cutaneous manifestations are very common in PIDs. As an easy accessible organ, skin canbecrucial for early diagnosis and treatment. This is relevant for preventing significant disease-associated morbidity and mortality...
August 9, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28774826/erythrocyte-based-drug-delivery-in-transfusion-medicine-wandering-questions-seeking-answers
#19
REVIEW
Vassilis L Tzounakas, Dimitrios G Karadimas, Issidora S Papassideri, Jerard Seghatchian, Marianna H Antonelou
Red blood cells (RBCs) represent the most commonly used and best-studied natural carriers in the history of drug delivery. Their abundance and long circulation half-life, their great immune-biocompatibility and biodegradability profiles, along with the availability of well established protocols for their safe collection, ex vivo processing and quality control make them advantageous as drug delivery systems (DDS). As a result, several drug-loading techniques (including encapsulation and surface conjugation) have been developed in order to construct RBC-based or RBC-inspired drug delivery vehicles for the effective treatment of infections, cancer, chronic and autoimmune diseases in both pre-clinical protocols and clinical trials...
July 16, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28756176/infection-mediated-autoimmune-movement-disorders
#20
Alberto Cucca, Hamzeh A Migdadi, Alessandro Di Rocco
Infectious diseases remain the most common cause of neurological disability in the world. A number of movement disorders can develop in adults and children in response to infections. These can occur in isolation or as part of a broader neurological illness, with movement abnormalities consequent to an encephalopathy or a broader brain dysfunction. While most infection-related movement disorders are direct consequences of an active infectious process affecting cerebral structures implied in the motor network, at times a delayed immune-mediated process in response to a previous infectious is responsible for the neurological dysfunction...
July 21, 2017: Parkinsonism & related Disorders
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