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Treatment of CVID

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https://www.readbyqxmd.com/read/29368942/autoimmune-hepatitis-as-a-complication-of-common-variable-immunodeficiency
#1
Patrícia de Oliveira Queirós, José Manuel Sousa Martín
In common variable immunodeficiency (CVID) there is a deregulation of the immune system, which frequently leads to an increased risk of infections, but also to autoimmunity phenomena. Autoimmune hepatitis may develop at any time of CVID's evolution, but it is difficult to diagnose due to the frequent absence of autoantibodies and low levels of IgG. Early diagnosis is important because targeted treatment may allow disease improvement. We present a case of autoimmune hepatitis in a patient with CVID.
January 25, 2018: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29325751/a-difficult-diagnosis-of-coeliac-disease-repeat-duodenal-histology-increases-diagnostic-yield-in-patients-with-concomitant-causes-of-villous-atrophy
#2
Gaetano Cristian Morreale, Luigi Maria Montalbano, Maria Cappello, Emanuele Sinagra, Aroldo Rizzo, Antonio Carroccio
Villous atrophy in absence of coeliac disease (CD)-specific antibodies represents a diagnostic dilemma. We report a case of a woman with anaemia, weight loss and diarrhoea with an initial diagnosis of seronegative CD and a histological documented villous atrophy who did not improve on gluten-free diet due to the concomitant presence of common variable immunodeficiency (CVID) and Giardia lamblia infection. This case report confirms that CD diagnosis in CVID patients is difficult; the combination of anti-endomysial antibodies (EmA-IgA), anti-tissue transglutaminase antibodies (tTG-IgAb) antibodies and total IgA is obligatory in basic diagnostic of CD but in CVID are negative...
December 2017: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
https://www.readbyqxmd.com/read/29277461/intra-cranial-granulomatous-disease-in-common-variable-immunodeficiency-case-series-and-review-of-the-literature
#3
REVIEW
Catherine E Najem, Jason Springer, Richard Prayson, Daniel A Culver, James Fernandez, Jinny Tavee, Rula A Hajj-Ali
BACKGROUND/PURPOSE: Common variable immunodeficiency (CVID) is typically characterized by hypogammaglobulinemia and often but not always recurrent infections. Paradoxically, 8-22% of patients with CVID develop granulomatous disease. Granulomata have been described in many organs including the lungs, skin, liver, spleen, kidneys, eyes, lymph nodes, and intestines. Data about central nervous system (CNS) involvement in CVID are extremely rare. We aim to describe a case series and include an extensive literature review of CNS involvement in CVID to understand the different features and patterns of the disease...
October 20, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29249107/-common-immunodeficiency-variable-in-adults
#4
Patricia María O'Farrill-Romanillos, Diana Andrea Herrera-Sánchez, Cecilia Hernández-Fernández, Eunice Giselle López-Rocha
Primary immunodeficiencies (PIDs) are low-incidence diseases caused by defects in genes involved in the development, maintenance, and regulation of the immune system. Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency of adulthood. It has an approximate prevalence of 1 in 25 000-50 000 in the general population, with a delay in diagnosis between 6-7 years. The clinical manifestations of CVID constitute six main categories: infections, pulmonary complications, granulomatous or polyclonal lymphocytic disease, autoimmunity, gastrointestinal diseases and malignancy Most patients must have at least one of the following clinical manifestations (infection, autoimmunity, lymphoproliferation)...
October 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/29225631/initial-intravenous-immunoglobulin-doses-should-be-based-on-adjusted-body-weight-in-obese-patients-with-primary-immunodeficiency-disorders
#5
Rohan Ameratunga
Background: Immunoglobulin therapy plays a critical role in the treatment of immunodeficiency disorders as well as autoimmune and inflammatory conditions. In immunodeficient patients, there has been controversy whether initial loading doses of intravenous (IVIG) should be based on actual body weight or a calculated parameter such as adjusted body weight in obese patients. Case presentation: I describe a patient with Common Variable Immunodeficiency disorder (CVID) who underwent bariatric surgery for morbid obesity...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/29184600/-common-variable-immune-deficiency-lately-revealed-by-gastrointestinal-problems-about-a-case
#6
Fatima Ezzaitouni, Youssef Thiyfa, Mohamed Tahiri, Fouad Haddad, Wafaa Hliwa, Ahmed Bellabah, Wafaa Badre
Common Variable Immune Deficiency (CVID) is rare. It is a constitutional deficit of humoral immunity characterized by recurrent bacterial infections and by increased frequency of tumors, autoimmune or granulomatous diseases. Gastrointestinal manifestations are very variable and sometimes reveal common variable immune deficiency. We report the case of a 31-year old patient with a history of childhood recurrent respiratory infections complicated by bronchiectasis and with a 3-year history of recurrent glairy diarrhea...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/29114494/giardiasis-mimicking-celiac-disease-in-a-patient-of-common-variable-immunodeficiency
#7
Kumar Saurabh, Vijaya Lakshmi Nag, Daisy Khera, Poonam Elhence
An adolescent boy presented to pediatrics outpatient department with complaints of recurrent diarrhea, nausea, vomiting, and pedal edema since 3-4 months, with no relief even after taking treatment. His investigation revealed decreased serum IgA, IgG, and IgM levels with hypoproteinemia. Duodenal biopsy showed features of celiac disease (CD), but tissue transglutaminase IgA was negative. In stool examination, plenty of Giardia lamblia cysts and eggs of Hymenolepis nana were present. He improved on treatment and remained asymptomatic for 4 months...
July 2017: Tropical Parasitology
https://www.readbyqxmd.com/read/28973009/tlr9-stimulation-of-b-cells-induces-transcription-of-p53-and-prevents-spontaneous-and-irradiation-induced-cell-death-independent-of-dna-damage-responses-implications-for-common-variable-immunodeficiency
#8
Kristine Lillebø Holm, Randi Gussgard Syljuåsen, Grete Hasvold, Lene Alsøe, Hilde Nilsen, Kristina Ivanauskiene, Philippe Collas, Sergey Shaposhnikov, Andrew Collins, Randi Larsen Indrevær, Pål Aukrust, Børre Fevang, Heidi Kiil Blomhoff
In the present study, we address the important issue of whether B-cells protected from irradiation-induced cell death, may survive with elevated levels of DNA damage. If so, such cells would be at higher risk of gaining mutations and undergoing malignant transformation. We show that stimulation of B-cells with the TLR9 ligands CpG-oligodeoxynucleotides (CpG-ODN) prevents spontaneous and irradiation-induced death of normal peripheral blood B-cells, and of B-cells from patients diagnosed with Common variable immunodeficiency (CVID)...
2017: PloS One
https://www.readbyqxmd.com/read/28937520/evans-syndrome-as-first-manifestation-of-primary-immunodeficiency-in-clinical-practice
#9
Libny Martínez-Valdez, Angela Deyà-Martínez, María T Giner, Rubén Berrueco, Ana Esteve-Solé, Manel Juan, Ana M Plaza-Martín, Laia Alsina
BACKGROUND: Evans syndrome (ES) is a rare immune disorder in children, manifested by simultaneous or sequential autoimmune cytopenias (ACs) of unknown cause and having a chronic course with periods of exacerbation and remission. Some primary immunodeficiencies (PIDs) may present with autoimmune manifestations without infections, masking suspicion of them. The PIDs that can typically manifest as ES are autoimmune lymphoproliferative syndrome and common variable immunodeficiency (CVID)...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28871669/facilitated-subcutaneous-immunoglobulin-fscig-in-autoimmune-cytopenias-associated-with-common-variable-immunodeficiency
#10
Veronica Pedini, Isabella Savore, Giovanna Maria Danieli
BACKGROUND: Common variable immunodeficiency (CVID) is the most common symptomatic primary immune deficiency of adulthood. Besides recurrent infections, autoimmune disorders-mainly cytopenias-affect 30% of patients with CVID. OBJECTIVES: To describe the efficacy and safety of facilitated subcutaneous immunoglobulin (fSCIg), which is a combination of 10% [human] SCIg with recombinant human hyaluronidase for the treatment of CVID-linked cytopenias. METHODS: We describe four women (mean age 54 years) with CVID associated with idiopathic thrombocytopenic purpura (ITP) (n=3) and autoimmune hemolytic anemia (AIHA) (n=1)...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28861081/14%C3%A2-years-after-discovery-clinical-follow-up-on-15-patients-with-inducible-co-stimulator-deficiency
#11
Johanna Schepp, Janet Chou, Andrea Skrabl-Baumgartner, Peter D Arkwright, Karin R Engelhardt, Sophie Hambleton, Tomohiro Morio, Ekkehard Röther, Klaus Warnatz, Raif Geha, Bodo Grimbacher
BACKGROUND: Inducible co-stimulator (ICOS) deficiency was the first monogenic defect reported to cause common variable immunodeficiency (CVID)-like disease in 2003. Since then, 16 patients have been reported worldwide with an increasing range of clinical phenotypes. OBJECTIVE: We sought to compare the clinical and immunological phenotype and provide clinical follow-up and therapeutic approaches for treating ICOS-deficient patients. METHODS: We describe the clinical and laboratory data of 15 patients with available clinical data...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28734862/respiratory-infections-and-antibiotic-usage-in-common-variable-immunodeficiency
#12
Johannes M Sperlich, Bodo Grimbacher, Sarita Workman, Tanzina Haque, Suranjith L Seneviratne, Siobhan O Burns, Veronika Reiser, Werner Vach, John R Hurst, David M Lowe
BACKGROUND: Patients with common variable immunodeficiency (CVID) suffer frequent respiratory tract infections despite immunoglobulin replacement and are prescribed significant quantities of antibiotics. The clinical and microbiological nature of these exacerbations, the symptomatic triggers to take antibiotics, and the response to treatment have not been previously investigated. OBJECTIVES: To describe the nature, frequency, treatment, and clinical course of respiratory tract exacerbations in patients with CVID and to describe pathogens isolated during respiratory tract exacerbations...
July 19, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28718689/orbital-diffuse-large-b-cell-lymphoma-with-combined-variable-immunodeficiency
#13
Vishal S Parikh, Deepa Jagadeesh, James M Fernandez, Eric D Hsi, Arun D Singh
Common variable immunodeficiency (CVID) is a primary immunodeficiency manifesting as a reduction in the level of total immunoglobulin (Ig) G, a reduction in the level of either IgA or IgM, poor response to polysaccharide vaccine, and usually frequent infections. The association of CVID with an increased risk of malignancy, specifically lymphoma, is well known. A 63-year-old female with a past medical history significant for CVID presented with a 1-month history of dull, left eye pain with proptosis, hypoglobus, and left upper lid fullness without a discrete palpable mass...
July 18, 2017: Orbit
https://www.readbyqxmd.com/read/28672090/efficacy-of-mycophenolate-on-lung-disease-and-autoimmunity-in-children-with-immunodeficiency
#14
Giorgia Bucciol, Angelamaria Petrone, Maria Caterina Putti
The autoimmune manifestations of primary immunodeficiencies, such as autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID), often constitute a great therapeutic challenge and have a significant impact on patients' morbidity and mortality. The most common autoimmune presentations are autoimmune cytopenias, but organ-related autoimmunity is also frequently observed. From a pulmonology perspective, granulomatous/lymphocytic interstitial lung disease (GLILD) is a severe immunological complication which significantly worsens the clinical outcome of these patients and for which there are currently few guidelines or protocols for treatment...
October 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28623282/a-nationwide-study-of-severe-and-protracted-diarrhoea-in-patients-with-primary-immunodeficiency-diseases
#15
Wen-I Lee, Chien-Chang Chen, Tang-Her Jaing, Liang-Shiou Ou, Chuen Hsueh, Jing-Long Huang
Diarrhoea lasting longer than 14 days and failing to respond to conventional management is defined as severe and protracted diarrhoea (SD). In this study, we investigated the prevalence, pathogens and prognosis of SD in primary immunodeficiency diseases (PIDs). Among 246 patients with predominantly paediatric-onset PIDs from 2003-2015, 21 [Btk (six), IL2RG (four), WASP, CD40L, gp91 (three each), gp47, RAG2 (one each)] and five [CVID (four), SCID (one)] without identified mutations had SD before prophylactic treatment...
June 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28606051/comparison-of-bone-mineral-density-in-common-variable-immunodeficiency-and-x-linked-agammaglobulinaemia-patients
#16
Ali Mohebbi, Gholamreza Azizi, Naeimeh Tavakolinia, Mehdi Karimipour, Fatemeh Kiaee, Reza Yazdani, Sareh Sadat Ebrahimi, Hosein Rafiemanesh, Vahid Ziaee, Hassan Abolhassani, Asghar Aghamohammadi, Farzaneh Abbasi, Fatemeh Sayarifard, Mehran Ebrahimi, Javad Tafaroji
BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders, resulting from different defects in development and function of B cell lineage. Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) are two of the major types of PADs. Optimal growth and subsequently bone health could potentially compromise due to the interference of several factors in PAD with childhood onset. In the present study, our aim was to evaluate bone mineral density (BMD) of patients with CVID and XLA...
June 11, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28600865/immune-deficiency-and-autoimmunity-in-patients-with-ctla-4-cd152-mutations
#17
REVIEW
N Verma, S O Burns, L S K Walker, D M Sansom
Immune deficiency disorders are a heterogeneous group of diseases of variable genetic aetiology. While the hallmark of immunodeficiency is susceptibility to infection, it is increasingly clear that autoimmunity is prevalent, suggestive of a more general immune dysregulation in some cases. With the increasing use of genetic technologies, the underlying causes of immune dysregulation are beginning to emerge. Here we provide a review of the heterozygous mutations found in the immune checkpoint protein CTLA-4, identified in cases of common variable immunodeficiency disorders (CVID) with accompanying autoimmunity...
October 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28554560/th1-phenotype-of-t-follicular-helper-cells-indicates-an-ifn%C3%AE-associated-immune-dysregulation-in-cd21low-cvid-patients
#18
Susanne Unger, Maximilian Seidl, Pauline van Schouwenburg, Mirzokhid Rakhmanov, Alla Bulashevska, Natalie Frede, Bodo Grimbacher, Jens Pfeiffer, Klaudia Schrenk, Luis Munoz, Leif Hanitsch, Ina Stumpf, Fabian Kaiser, Oliver Hausmann, Florian Kollert, Sigune Goldacker, Mirjam van der Burg, Baerbel Keller, Klaus Warnatz
BACKGROUND: A subgroup of patients with Common Variable Immunodeficiency (CVID) suffers from immune dysregulation, manifesting as autoimmunity, lymphoproliferation and organ inflammation, and thereby increasing morbidity and mortality. Therefore, treatment of these complications demands a deeper comprehension of their etiology and pathophysiology. OBJECTIVES: Based on the identification of an Interferon (IFN) signature in CVID patients with secondary complications and a skewed T follicular helper cell (TFH) differentiation in defined monogenic immunodeficiencies, we sought to determine the profile of CD4 memory T cells in blood and secondary lymphatic tissues of these patients...
May 26, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28536745/health-related-quality-of-life-in-adult-patients-with-common-variable-immunodeficiency-disorders-and-impact-of-treatment
#19
Nicholas L Rider, Carleigh Kutac, Joud Hajjar, Chris Scalchunes, Filiz O Seeborg, Marcia Boyle, Jordan S Orange
PURPOSE: Common variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. However, how Ig treatment in CVID affects quality of life (QOL) has not been thoroughly evaluated. The purpose of a recent Immune Deficiency Foundation (IDF) mail survey was to assess the factors that are associated with QOL in patients with CVID receiving Ig treatment...
July 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28433201/humoral-primary-immunodeficiency-diseases-clinical-overview-and-chest-high-resolution-computed-tomography-hrct-features-in-the-adult-population
#20
REVIEW
L Cereser, R Girometti, P d'Angelo, M De Carli, A De Pellegrin, C Zuiani
Humoral primary immunodeficiency diseases (hPIDs) are a heterogeneous group of hereditary disorders resulting in abnormal susceptibility to infections of the sinopulmonary tract. Some of these conditions (e.g., common variable immunodeficiency disorders [CVID]) imply a number of non-infectious thoracic complications such as non-infectious airway disorders, diffuse lung parenchymal diseases, and neoplasms. Chest high-resolution computed tomography (HRCT) is a key imaging tool to characterise and quantify the extent of underlying thoracic involvement, as well as to direct and monitor treatment...
July 2017: Clinical Radiology
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