Journal articles: 'Common variable immunodeficiency (CVID)'

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Suter-Meyer, Nigg, Kolyvanos Naumann, Käser, and Vetter. "Common Variable Immunodeficiency (CVID) – variables humorales Immundefektsyndrom." Praxis 96, no. 1 (January 1, 2007): 3–11. http://dx.doi.org/10.1024/1661-8157.96.1.3.

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Vukas, Emina, Aida Dizdarević, Senka Mesihović - Dinarević, and Adisa Čengić. "Common variable immunodeficiency – case report." Journal of Health Sciences 3, no. 2 (September 15, 2013): 170–72. http://dx.doi.org/10.17532/jhsci.2013.83.

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Common variable immunodeficiency (CVID) or acquired hypogammaglobulinemia is the type of primary immunodeficiency. Deregulation of the immune system, leading to hypogammaglobulinemia, defective activation and proliferation of T cells and dendritic cells, and malfunction of the cytokines are observed in CVID. The clinical picture of CVID varies, any organ or system can be affected, therefore the diagnosis is often difficult and delayed and sometimes is not always possible. This article describes a twelve years old boy with all the clinical signs of immunodeficiency, as confi rmed by laboratory. The main treatment consists of life-long immunoglobulin substitution in intravenous or subcutaneous form.

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Capistrano, Giovany Gomes, Gdayllon Cavalcante Meneses, Fernanda Macedo de Oliveira Neves, Renata de Almeida Leitão, Alice Maria Costa Martins, and Alexandre Braga Libório. "Renal Evaluation in Common Variable Immunodeficiency." Journal of Immunology Research 2018 (2018): 1–6. http://dx.doi.org/10.1155/2018/5841031.

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Introduction. Common variable immunodeficiency (CVID) comprises a heterogeneous group of disorders characterized by impaired antibody production. Kidney involvement in CVID is described in isolated and sporadic case reports. The objective of this study was to study the renal function pattern in CVID patients through glomerular and tubular function tests. Methods. Study of 12 patients with CVID diagnosis and 12 healthy control individuals. Glomerular filtration rate (GFR), fractional excretion of sodium (FENa+) and potassium (FEK+), urinary concentration, and acidification capacity were measured. In addition, microalbuminuria and urinary monocyte chemoattractant protein-1 (MCP-1) were evaluated as markers of selectivity of the glomerular barrier and inflammation, respectively. Results. In relation to glomerular markers, all CVID patients had normal GFR (>90 mL/min/1.73 m2), and microalbuminuria and urinary MCP-1 levels were also similar to those of controls. Interestingly, CVID patients had reduced urinary concentration capacity, as demonstrated by lower U/POsm ratio, when compared to controls. Also, while all control subjects achieved a urinary pH less than 5.3, no CVID patients showed a decrease in urinary pH to such levels in response to acid loading with CaCl2, characterizing impaired urinary acidification capacity. Conclusion. Patients showed a trend towards an elevated prevalence of tubular dysfunction, mainly related to urinary acidification and concentration capacities.

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Caza, Tiffany N., Samar I. Hassen, and Christopher P. Larsen. "Renal Manifestations of Common Variable Immunodeficiency." Kidney360 1, no. 6 (April 21, 2020): 491–500. http://dx.doi.org/10.34067/kid.0000432020.

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BackgroundCommon variable immunodeficiency (CVID) is one of the most common primary immunodeficiency syndromes, affecting one in 25,000–50,000 people. Renal insufficiency occurs in approximately 2% of patients with CVID. To date, there are no case series of renal biopsies from patients with CVID, making it difficult to determine whether individual cases of renal disease in CVID represent sporadic events or are related to the underlying pathophysiology. We performed a retrospective analysis of renal biopsy specimens in our database from patients with a clinical history of CVID (n=22 patients, 27 biopsies).MethodsLight, immunofluorescence, and electron microscopy were reviewed. IgG subclasses, PLA2R immunohistochemistry, and THSD7A, EXT1, and NELL1 immunofluorescence were performed on all membranous glomerulopathy cases. CD3, CD4, CD8, and CD20 immunohistochemistry was performed on cases of tubulointerstitial nephritis.ResultsAKI and proteinuria were the leading indications for renal biopsy in patients with CVID. Immune-complex glomerulopathy was present in 12 of 22 (54.5%) cases, including nine cases with membranous glomerulopathy, one case with a C3 glomerulopathy, and one case with membranoproliferative GN with IgG3κ deposits. All membranous glomerulopathy cases were PLA2R, THSD7A, EXT1, and NELL1 negative. The second most common renal biopsy diagnosis was chronic tubulointerstitial nephritis, affecting 33% of patients. All tubulointerstitial nephritis cases showed tubulitis and a lymphocytic infiltrate with >90% CD3+ T cells. Other renal biopsy diagnoses within our cohort included acute tubular injury (n=1), amyloid light-chain amyloidosis (n=1), diabetic glomerulosclerosis (n=1), thin basement membranes (n=1), pauci-immune GN (n=1), and arterionephrosclerosis (n=1).ConclusionsMembranous glomerulopathy and tubulointerstitial nephritis were the predominant pathologic findings in patients with CVID. Membranous glomerulopathy cases in patients with CVID were IgG1 subclass dominant and showed mesangial immune deposits. Four of the membranous glomerulopathy cases had associated proliferation, with mesangial and/or endocapillary hypercellularity, with or without crescent formation. CVID should be considered as a potential cause when membranous glomerulopathy or chronic tubulointerstitial nephritis is seen in a young patient with a history of recurrent infections.

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Tanir Basaranoglu, Sevgen, Sukru Cekic, Emine Kirhan, Melahat Dirican, and Sara S. Kilic. "Oxidative stress in common variable immunodeficiency." European Journal of Inflammation 19 (January 2021): 205873922110024. http://dx.doi.org/10.1177/20587392211002411.

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Common variable immunodeficiency (CVID) is a heterogenous group of immunologic disorders of unknown etiology. Alterations of the normal cellular balance due to an increase in reactive oxygen species and/or decrease in antioxidant defense may lead to increased oxidative stress. We aimed to evaluate the levels of oxidative stress biomarkers in patients with CVID who had different presentations. We investigated the serum catalase (CAT), erythrocyte superoxide dismutase (SOD), erythrocyte reduced glutathione as antioxidants and serum malondialdehyde levels as lipid peroxidation marker in patients with CVID in Uludag University Hospital Department of Pediatric Allergy and Immunology’s outpatient clinics. In the analysis, there were 21 patients and 27 matched healthy controls. The median levels of CAT in patients with CVID was significantly lower than in healthy controls ( p = 0.04). Among the patients with CVID, 19% had autoimmune disease, one had Sjögren’s syndrome, one had autoimmune alopecia, one had juvenile rheumatoid arthritis, and one had chronic inflammatory demyelinating polyneuropathy. Patients with autoimmune complications had significantly lower CAT levels compared to the ones without autoimmune diseases ( p = 0.03). The patients without non-infectious complications (NICs) had lower SOD levels than the patients with NICs ( p = 0.05). The analysis of oxidative stress markers in the patients with CVID suggested a series of abnormalities in the anti-oxidant system. The clinical syndrome associations may be a useful tool for future studies to set prediction markers for the prognosis of patients with CVID.

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Akkinepally, Sudha, Christian Mendez, and Vijayarama Poreddy. "Giardiasis in Common Variable Immunodeficiency (CVID)." American Journal of Gastroenterology 105 (October 2010): S247—S248. http://dx.doi.org/10.14309/00000434-201010001-00685.

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Coraglia, Ana, Nora Galassi, Diego S. Fernández Romero, M. Cecilia Juri, Marta Felippo, Alejandro Malbrán, and María M. E. de Bracco. "Common Variable Immunodeficiency and Circulating TFH." Journal of Immunology Research 2016 (2016): 1–10. http://dx.doi.org/10.1155/2016/4951587.

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CD4+ T follicular helper cells (TFH) were assessed in adult patients with common variable immune deficiency (CVID) classified according to the presence of granulomatous disease (GD), autoimmunity (AI), or both GD and AI (Group I) or the absence of AI and GD (Group II).TFHlymphocytes were characterized by expression of CXCR5 and PD-1.TFHwere higher (in both absolute number and percentage) in Group I than in Group II CVID patients and normal controls (N). Within CXCR5+CD4+ T cells, the percentage of PD-1 (+) was higher and that of CCR7 (+) was lower in Group I than in Group II and N. The percentages of Treg andTFHreg were similar in both CVID groups and in N.TFHresponded to stimulation increasing the expression of the costimulatory molecules CD40L and ICOS as did N. After submitogenic PHA+IL-2 stimulation, intracellular expression ofTFHcytokines (IL-10, IL-21) was higher than N in Group I, and IL-4 was higher than N in Group II. These results suggest thatTFHare functional in CVID and highlight the association of increased circulatingTFHwith AI and GD manifestations.

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Ellwood, Amanda, and Caroline Hamm. "Common Variable Immunodeficiency Presenting with Hodgkin’s Disease." Blood 106, no. 11 (November 16, 2005): 4642. http://dx.doi.org/10.1182/blood.v106.11.4642.4642.

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Abstract We report a case of a 32 year old man with classic Common Variable Immunodeficiency Disease (CVID) and Hodgkin’s disease. Repeated immunoglobulin studies as a child showed hypogammaglobulinemia and decreased mitogen responses, with the suggestion of CVID. He presented to the oncology clinic with nearly three decades of recurrent reactive lymphadenopathy and multiple lymph node biopsies throughout his lifetime, recurrent pulmonary infections, and multiple autoimmune manifestations including diabetes mellitus onset at age 4, autoimmune hemolytic anemia and autoimmune thrombocytopenia. He developed Hodgkin’s disease at the age of 30, which has not previously been described in CVID. We also report two other cases of CVID with the diagnosis of non-Hodgkin’s lymphoma in the same oncology practice. CVID is a relatively common and yet rarely identified syndrome. Specific treatment is recommended. We suggest that oncologists should be aware of this unusual and yet relatively common disorder that carries up to 400 times the risk for the development of lymphoma (Cunningham-Rundles, C, et al. J Clin Immunol1987; 7:294–299).

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Pecoraro, Heidi L., M. Julia B. Felippe, Andrew D. Miller, Thomas J. Divers, Kenneth W. Simpson, Kimberly M. Guyer, and Gerald E. Duhamel. "Neuroborreliosis in a horse with common variable immunodeficiency." Journal of Veterinary Diagnostic Investigation 31, no. 2 (January 19, 2019): 241–45. http://dx.doi.org/10.1177/1040638718824146.

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Common variable immunodeficiency (CVID) is a rare condition in adult horses characterized by hypogammaglobulinemia and increased susceptibility to parasitic and bacterial infections, including recurrent respiratory diseases, septicemia, and meningitis. Lyme disease is often included as a differential diagnosis in CVID horses with signs of meningitis; however, the Borrelia burgdorferi organism has not been demonstrated previously within central nervous system tissues of CVID horses with neurologic disease, to our knowledge. We report herein a case of neuroborreliosis in a CVID horse, confirmed by combined immunologic testing, histopathology, real-time PCR assay, fluorescent in situ hybridization, and immunohistochemical staining. Implications of these findings include heightened monitoring of CVID horses for Lyme disease in endemic areas and appropriate therapy in the case of neurologic disease.

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Montazeri, Maryam, Mohammad Reza Ebadpour, Farideh Kouchak, and Naser Esmaeili. "Common Variable Immunodeficiency (CVID): A Case Report." Internal Medicine and Medical Investigation Journal 2, no. 1 (March 20, 2017): 26. http://dx.doi.org/10.24200/imminv.v2i1.44.

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Common variable immune deficiency disease is the most prevalent acquired immune deficiency in human being after selective immunoglobulin A deficiency. It causes reduction of immunoglobulin levels and specific antibodies production and enhancement of recurrent and chronic infections risk, especially respiratory infections. CVID patients faces increased risk of granulomatous disease, autoimmune and phenomenon and malignancy. The disease involves males and females equally. Some studies showed that early diagnosis of CVID disease and regular treatment of patients with IVIG may have an efficient role in decreasing pneumonia and frequency of hospitalization due to infections and its complications. In this study we report a 16 years old girl with CVID, without clinical history of determined infection with recurrent sinusitis.

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Fernandes, Karin Sá, Michella Bezerra Lima, Cíntia de Paula Martins, Maria Cristina dos-Santos, Fabio Daumas Nunes, Cristina Maria Kokron, and Marina Gallottini. "Salivary Immunoglobulins in Individuals with Common Variable Immunodeficiency." Brazilian Dental Journal 27, no. 6 (December 2016): 641–45. http://dx.doi.org/10.1590/0103-6440201601096.

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Abstract Oral manifestations of common variable immunodeficiency (CVID) are rare, have rarely been studied and have given controversial results. There are few data about IgA, IgG, and IgM antibody salivary levels in the literature, and there are few papers about the clinical impact of antibody deficiencies and CVID on the oral health of such patients. The aim of this study was to measure serum and salivary IgA, IgG, and IgM levels in CVID participants and controls, and to associate immunoglobulin levels with caries and periodontal disease. This was a case-control study involving 51 CVID individuals and 50 healthy controls. All participants underwent examination for dental caries and periodontal disease. Blood and whole saliva samples were collected on the same day of the oral examination. Serum IgA, IgM, and IgG levels were measured by turbidimetry and salivary IgA, IgM, and IgG titers were assessed by enzyme-linked immunosorbent assay. Incidences of caries and gingivitis were significantly higher in the CVID group than in the control group (p<0.05). Salivary and blood IgA and IgM titers were significantly reduced in the CVID group, but there was no association of salivary immunoglobulin levels with periodontal disease or with caries incidence (p>0.05 for both). Although CVID was associated with increased susceptibility to caries and gingivitis, it was not associated with low salivary levels of IgA and IgM.

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Cunningham-Rundles, Charlotte. "Common variable immune deficiency: case studies." Blood 134, no. 21 (November 20, 2019): 1787–95. http://dx.doi.org/10.1182/blood.2019002062.

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In this review, the authors describe 3 patients with common variable immunodeficiency (CVID), noting the disease manifestations most relevant to the practicing hematologist, especially autoimmune cytopenias, benign lymphoproliferation, granulomatous disease, and lymphomas such as common noninfectious complications of CVID.

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Bonilla, Francisco A. "Personalized therapy for common variable immunodeficiency." Allergy and Asthma Proceedings 41, no. 1 (January 1, 2020): 19–25. http://dx.doi.org/10.2500/aap.2020.41.190012.

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Background: Common variable immunodeficiency (CVID) represents a clinical descriptive diagnosis that was defined in the 1970s. Despite the vast increase in knowledge with regard to immune function and genetics, the pathophysiology of this disorder remains poorly understood in the majority of patients (75%); however, recent advances have led to a much clearer understanding of this heterogeneous group of disorders in the remaining 25%. These advances, along with developments in immune modulatory and reconstitution therapies, now permit sophisticated and specific targeting of therapies for individual patients. Methods: A literature review and author experience. Results: For > 50 years, immune globulin therapy has been applied to patients with CVID. There are several options open to patients, including a diversity of products and modes of administration. Stem cell therapy is increasingly applicable in patients with severe immune dysregulation. In some disorders (e.g., lipopolysaccharide-responsive and beige-like anchor protein, and cytotoxic T lymphocyte antigen 4 deficiencies), knowledge of the genetic basis and molecular pathophysiology permit targeted therapy by using small-molecule immune modulators and biologics. Conclusion: In the near future, it is likely that further advances in understanding the pathophysiology of CVID, together with ongoing development of biologics and small-molecule immune modulators will lead to additional targeted therapies for these patients.

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Speth, F. "Common Variable Immunodeficiency (CVID) und therapierefraktäre Polyarthritis." Arthritis und Rheuma 32, no. 05 (2012): 330–33. http://dx.doi.org/10.1055/s-0037-1618142.

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Spickett, G. P. "Current perspectives on common variable immunodeficiency (CVID)." Clinical & Experimental Allergy 31, no. 4 (April 2001): 536–42. http://dx.doi.org/10.1046/j.1365-2222.2001.01117.x.

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Mathew, S. "Thrombocytopenia associated with common variable immunodeficiency (CVID)." Journal of Allergy and Clinical Immunology 115, no. 2 (February 2005): S159. http://dx.doi.org/10.1016/j.jaci.2004.12.648.

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Nasa, Mukesh, Smruti Ranjan Mishra, Lipika Lipi, and Randhir Sud. "Common variable immunodeficiency syndrome with chronic diarrhoea." BMJ Case Reports 12, no. 3 (March 2019): e228240. http://dx.doi.org/10.1136/bcr-2018-228240.

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Common variable immunodeficiency syndrome (CVID) is a heterogeneous disorder characterised by diminished levels of IgG, IgA and/or IgM, and recurrent bacterial infections. Sinopulmonary infections are most commonly reported followed by gastrointestinal (GI) infections. GI tract represents the largest immune organ with abundance of lymphoid cells, its involvement can manifest variably ranging from asymptomatic involvement to florid symptoms and signs. Diffuse nodular lymphoid hyperplasia (DNLH) of the GI tract is characterised by numerous small polypoid nodules of variable size in the small intestine, large intestine or both. It is commonly seen in association to immunodeficiency states such as CVID, IgA deficiency and chronic infections due to Giardia lamblia and Helicobacter pylori and cryptosporidiosis. Repetitive antigenic stimulation leads to lymphoid hyperplasia. We herein describe a case of DNLH of the intestine and another case of duodenal cytomegalovirus (CMV) infection associated with CVID.

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Huq, Madiha, Neha Sanan, Phuong Daniels, and Robert Hostoffer. "Posner-Schlossman Syndrome in Common Variable Immunodeficiency." Case Reports in Ophthalmological Medicine 2020 (October 15, 2020): 1–3. http://dx.doi.org/10.1155/2020/8843586.

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Introduction. Posner-Schlossman syndrome (PSS) is a rare glaucomatocyclitic crisis with clinical features including recurrent episodes of unilateral elevated intraocular pressure. Autoimmune and infectious causes have been proposed as potential etiologies of PSS. We report the first case of PSS in the setting of common variable immunodeficiency (CVID). Case Report. A sixty-two-year-old Caucasian female with a medical history of CVID and ulcerative colitis presented to the emergency room with complaints of acute right-sided vision changes. She reported image distortion, blurriness, and loss of central vision. Physical exam was significant for mildly injected right conjunctiva, visual acuity of 20/70 in right eye, and 20/25 in left eye. The right intraocular pressure was measured at 34 mmHg and left at 12 mmHg. The gonioscopy and dilated fundus examination were unremarkable. Cup to disc ratio was within normal limits, and no afferent pupillary defects were recorded. The patient was acutely treated with three rounds of dorzolamide/timolol and 0.2% brimonidine which decreased the right eye intraocular pressure to 24 mmHg. On follow-up exam with an ophthalmologist, anterior uveitis including an elevated pressure of 41 mmHg on the right and 18 mmHg on the left eye was noted and a PSS diagnosis was confirmed. Conclusion. PSS remains a rare condition with uncertain etiology and no associated systemic conditions. PSS has been postulated to be linked to autoimmune conditions. CVID is associated with many autoimmune disorders including Sjogren’s, rheumatoid arthritis, and colitis. There have been a few reported CVID-associated ocular diseases including granulomatous uveitis and conjunctivitis, chronic anterior uveitis, and birdshot retinopathy. We describe the first case of PSS in a patient with CVID.

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Shabashova, N. V., L. V. Filippova, A. E. Uchevatkina, and E. V. Frolova. "Common variable immunodeficiency in adults." Terapevticheskii arkhiv 88, no. 11 (November 15, 2016): 94–98. http://dx.doi.org/10.17116/terarkh2016881194-98.

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The paper analyzes 7 cases of common variable immune deficiency (CVID), a primary immunodeficiency disease. All the cases were detected in outpatients over the age of 40 years. The diagnosis was based on their history data and general clinical findings with due regard for the results of previously conducted functional studies, expert opinions, and the results of immunological studies including the quantitative and functional indices of T and B cells, phagocytes and the levels of immunoglobulins. The analysis showed that the early signs of impaired immunity in all the patients were seen by physicians of various specialties in both outpatient and inpatient settings. Generalizing of all information about the patient could become the basis for a simple and accessible practical public-health study of immunoglobulins levels significantly sooner than this diagnosis being verified. This testifies that the physicians of various specialties are partially aware of the clinical signs of immunodeficiency states and that there is a need for a clinical immunologist in adult healthcare facilities. This is especially important since the early clinical manifestations of both primary immunodeficiency disorders that are increasingly frequently detected and nonhereditary – secondary ones can be very similar. The timely verification of the diagnosis is necessary for prescribing adequate therapy with intravenous immunoglobulins to prevent severe chronic pyoinflammatory lung disease and disability in patients with CVID.

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Ricciardi, Luisa, Fabiana Furci, Antonio Ieni, and Antonio Macrì. "Castleman Disease in a Patient with Common Variable Immunodeficiency." Case Reports in Immunology 2019 (February 14, 2019): 1–5. http://dx.doi.org/10.1155/2019/5476383.

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Common variable immunodeficiency (CVID) is a primary immunodeficiency due to a disorder of the adaptive immune system which causes hypogammaglobulinemia and therefore an increased susceptibility to infection; noninfectious, inflammatory conditions including systemic autoimmunity and lymphoproliferative complications are also commonly associated with CVID. Castleman disease (CD) is a systemic disease clinically characterized by diffuse lymphadenopathy, splenomegaly, anemia, and systemic inflammatory symptoms. This makes CD a great mimicker of more common benign and malignant masses in the neck, chest, abdomen, and pelvis. A novel case of primary immunodeficiency (CVID) in a middle-aged woman, who developed multicentric CD (MDC) with splenomegaly, is described. The authors suggest that the onset of MCD and of the correlated splenomegaly was due to incorrect management of the hypogammaglobulinemia as immunoglobulin G (IgG) levels were not kept within normal ranges. Correct management of the hypogammaglobulinemia allowed splenectomy to be performed without any infectious surgical complications. MCD is reported for the first time in association with an adult case of CVID. The above reported case highlights the need for a timely correct diagnosis and treatment of CVID to avoid complications, which could cause recourse to splenectomy, such as in our case or development of malignancies.

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Silva, Gisele Barbosa e., Karla Pereira Fernandes, and Gesmar Rodrigues Silva Segundo. "Common variable immunodeficiency and isosporiasis: first report case." Revista da Sociedade Brasileira de Medicina Tropical 45, no. 6 (December 2012): 768–69. http://dx.doi.org/10.1590/s0037-86822012000600023.

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We report a severe case of diarrhea in a 62-year-old female HIV-negative patient from whom Giardia lamblia and Isospora belli were isolated. Because unusual and opportunistic infections should be considered as criteria for further analysis of immunological status, laboratory investigations led to a diagnosis of common variable immunodeficiency (CVID). This is the first reported case of isosporiasis in a patient with CVID and illustrates the importance of being aware of a possible link, particularly in relation to primary immunodeficiency.

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Holm, Are Martin, Pål Aukrust, Jan Kristian Damås, Fredrik Müller, Bente Halvorsen, and Stig S. Frøland. "Abnormal interleukin-7 function in common variable immunodeficiency." Blood 105, no. 7 (April 1, 2005): 2887–90. http://dx.doi.org/10.1182/blood-2004-06-2423.

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AbstractCommon variable immunodeficiency (CVID) is characterized by low levels of circulating immunoglobulins, leading to frequent infections, particularly of the respiratory tract. Frequently, T-cell abnormalities are observed. Interleukin-7 (IL-7) is involved in the homeostasis of lymphocytes, and may be elevated in lymphopenia. Mutations of genes related to IL-7 may lead to severe immunodeficiency disorders. We report elevated plasma levels of circulating IL-7 in a subgroup of CVID. These patients have increased numbers of circulating CD8+ T cells with decreased apoptosis and a predominance of CC chemokine receptor 7- (CCR7-) effector-memory T cells. Moreover, in some of these patients there is impaired response to IL-7 as assessed by in vitro proliferation and secretion of interferon γ and transforming growth factor β. These findings suggest novel pathogenic mechanisms and specific targets for further research in CVID.

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Khan, Mohammad Zahirul Islam, Kamrul Laila, Mohammed Mahbubul Islam, Mohammad Imnul Islam, and Shahana Akhter Rahman. "Common variable immunodeficiency disorder associated with bronchiectasis: a case report." International Journal of Contemporary Pediatrics 8, no. 3 (February 23, 2021): 565. http://dx.doi.org/10.18203/2349-3291.ijcp20210665.

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Common variable immunodeficiency disorder (CVID) is the commonest type of primary immunodeficiency disorders (PIDs) characterized by hypogammaglobulinemia, defective specific antibody production and increased susceptibility of recurrent infections. Autoimmunity, neoplasm and lymphoproliferative disorders are usually associated with CVID. In most cases, the cause is unknown, but multiple gene mutations (10%) may be associated with CVID. Here, we report an eight years old girl with CVID presented with recurrent infections, growth failure, generalized lymphadenopathy and hepatosplenomegaly. Chest examination and radiological findings of this girl were consistent with bronchiectasis. Lack of awareness among health care providers is the reason for delayed diagnosis of several years for this girl. Therefore, it is essential to raise awareness regarding PID patients among the physicians to improve the quality of life.

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Barmettler, Sara, Jocelyn Farmer, Alexandra Brady, Jolan E. Walter, and Mark Cobbold. "Gastrointestinal (GI) Manifestations in Common Variable Immunodeficiency (CVID)." Journal of Allergy and Clinical Immunology 141, no. 2 (February 2018): AB82. http://dx.doi.org/10.1016/j.jaci.2017.12.264.

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Shribman, Samuel E., Jessica Katanga, Nadeem Ali, Grant R. Hayman, Leslie R. Bridges, Maximillian S. Habibi, Andrew D. Mackinnon, and Arani Nitkunan. "Encephalomyelitis with Retinopathy in Common Variable Immunodeficiency (CVID)." Neuro-Ophthalmology 44, no. 1 (November 13, 2018): 38–40. http://dx.doi.org/10.1080/01658107.2018.1542008.

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Patuzzo, Giuseppe, Alessandro Barbieri, Elisa Tinazzi, Dino Veneri, Giuseppe Argentino, Francesca Moretta, Antonio Puccetti, and Claudio Lunardi. "Autoimmunity and infection in common variable immunodeficiency (CVID)." Autoimmunity Reviews 15, no. 9 (September 2016): 877–82. http://dx.doi.org/10.1016/j.autrev.2016.07.011.

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Grossman, William J., Patrick O. Bonnet, and Yan Xiong. "Increased Prevalence of Neoplasms In Patients Diagnosed with Common Variable Immunodeficiency." Blood 116, no. 21 (November 19, 2010): 1531. http://dx.doi.org/10.1182/blood.v116.21.1531.1531.

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Abstract Abstract 1531 Introduction: Common variable immunodeficiency (CVID) is a heterogenous disorder characterized by hypogammaglobulinemia, poor specific antibody responses, and increased numbers of infections. In addition, patients with CVID have been reported to carry an increased risk of developing other co-morbidities, including autoimmunity and certain types of neoplasms. Data establishing the overall risk and prevalence of these co-morbidities in patients with CVID has been limited due to the small sizes of patient cohorts examined to-date, and the lack of comprehensive primary immunodeficiency registries. The objective of this study was to document the prevalence rates of lymphoma select neoplasms in a large cohort of patients with CVID. Methods: The MedStat MarketScan database, which contains approximately 48 million patients, was evaluated for the number of IVIG-treated CVID patients using the 279.06 ICD-9 code for CVID over a 6-year period (2002-2008). Ten-fold the number of randomly selected patients, based on age, gender and length of enrollment, were used for matched control comparisons to our CVID cohort. Each cohort was evaluated for the rate of select neoplasms using ICD-9 codes for malignant lymphatic and hematopoietic tissue neoplasms (ICD-9 codes 200 to 208). Results: 814 CVID patients with at least 18 months of data were identified in the database, with 61% being female and 39% being male. The average age for females and males was 45 and 37 years, respectively (p<.0001). The CVID cohort carried a significantly higher prevalence of having a diagnosis code for a malignant neoplasm of the lymphatic and hematopoietic tissue than the control cohort [10.1% vs. 0.6%, respectively (p<.0001)]. Most of these neoplasm diagnoses occurred after the diagnosis of their CVID; however, 26.8% of neoplasm diagnoses occurred before the diagnosis of CVID. Additionally, the rates for each of the select neoplasms examined showed a significantly higher rate in the CVID cohort than in the control cohort. Conclusions: This study showed that CIVD patients have a higher prevalence of being co-diagnosed with certain neoplasms than matched controls. These data also suggest that CVID patients should not only be evaluated longitudinally for the development of neoplasms, but that certain cancer patients may also need to be considered and evaluated for underlying immune deficiencies such as CVID. Disclosures: Grossman: Baxter BioScience: Employment. Bonnet:Baxter BioScience: Employment. Xiong:BioScience: Employment.

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Kokron, Cristina M., Paolo R. Errante, Myrthes T. Barros, Gisele V. Baracho, Maristela M. Camargo, Jorge Kalil, and Luiz V. Rizzo. "Clinical and laboratory aspects of common variable immunodeficiency." Anais da Academia Brasileira de Ciências 76, no. 4 (December 2004): 707–26. http://dx.doi.org/10.1590/s0001-37652004000400007.

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Common variable immunodeficiency (CVID) is an immunological disorder characterized by defective antibody production, recurrent infections, most notably of the respiratory tract, autoimmune phenomena and cancer. Some CVID patients may also present disturbances of the cellular immune response such as a decrease in the number and proportion of different lymphocyte populations, diminished lymphoproliferative response to mitogens and antigens, altered production of cytokines, and deficient expression of cell-surface molecules. Most Brazilian CVID patients included in this study show a decrease in T and B lymphocyte counts in the peripheral blood. Furthermore, their lymphocytes are more susceptible to apoptosis following activation than normal individuals, and they have a decrease in the expression of activation molecules like CD25, CD69, CD40L and CD70. Moreover, they show a decreased synthesis of IL-4 and IL-5 in comparison with normal individuals. The increase in susceptibility to apoptosis following activation, may also be responsible for the decrease in the expression of activation molecules and CD40L, decrease in Th2 cytokines synthesis, and in the number of T and B circulating cells. In this study we discuss some of these immunological disturbances correlating them to the patients' clinical features and comparing our patients' findings to the literature.

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Litzman, Jiří, Zita Chovancová, Petr Bejdák, Marek Litzman, Zdeněk Hel, and Marcela Vlková. "Common variable immunodeficiency patients display elevated plasma levels of granulocyte activation markers elastase and myeloperoxidase." International Journal of Immunopathology and Pharmacology 33 (January 2019): 205873841984338. http://dx.doi.org/10.1177/2058738419843381.

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Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency diseases characterized by hypogammaglobulinemia and dysfunctional immune response to invading pathogens. Previous studies have indicated that CVID is associated with microbial translocation and systemic myeloid cell activation. The goal of this study was to determine whether patients with CVID display elevated systemic levels of markers of granulocyte activation and whether the levels are further influenced by intravenous immunoglobulin (IVIg) infusions. The plasma levels of granulocyte activation markers elastase and myeloperoxidase were determined using enzyme-linked immunosorbent assay (ELISA) in 46 CVID patients and 44 healthy controls. All CVID patients were in a stable state with no apparent acute infection. In addition, granulocyte activation markers’ plasma levels in 24 CVID patients were determined prior to and 1 h following IVIg administration. Neutrophil elastase and myeloperoxidase plasma levels were significantly higher in CVID patients than in healthy controls. Systemic elastase levels were further increased following IVIg administration. In vitro stimulation of 13 CVID patients’ whole blood using IVIg in a therapeutically relevant dose for 2 h resulted in a significant increase in plasma elastase levels compared to unstimulated blood. The data presented here indicate that CVID is associated with chronic granulocytic activation which is further exacerbated by administering IVIg. Increased myeloperoxidase and elastase levels may contribute to associated comorbidities in CVID patients.

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Aytekin, Gökhan, Fatih Çölkesen, Eray Yıldız, Mitat Arıcıgil, and Şevket Arslan. "Hearing Assessment in Adult Patients with Common Variable Immunodeficiency." Asthma Allergy Immunology 19, no. 1 (April 30, 2021): 38–45. http://dx.doi.org/10.21911/aai.600.

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ABSTRACT Objective: Common Variable Immunodeficiency (CVID) is a primary antibody disorder characterized by impaired B cell differentiation. Patients commonly present with acute and chronic sinusitis as well as otitis media, which may lead to hearing loss. Materials and Methods: Thirty-three CVID patients (20 male /13 female) with a mean age of 35 years (range 19-65 years) and 33 healthy individuals as a control group were included. Results: Among CVID patients, 17 (51.5%) had conductive hearing loss (CHL) , being unilateral in 4 (12.1%) and bilateral in 13 (39.4%). Unilateral and bilateral sensorineural hearing loss (SNHL) were detected in 2 (6.1%) and 5 (15.2%) respectively. CD4/CD8 cell ratio was significantly lower while CD8+ T lymphocyte ratio was significantly higher in those with CHL than in those without it (p=0.045 and p=0.009). Elevated CD8+ T cell ratio was an independent risk factor for CHL (p=0.015). Patients with SNHL were significantly older than those without it (p=0.040). CD16-56+ cell count was significantly lower in those with SNHL (p=0.031). Conclusion: CVID patients have an increased occurrence of CHL and SNHL, regardless of the cause. They provide evidence for the notion that these two types of hearing loss are not unrelated, immune dysregulation also plays a role in the process, and SNHL is not independent of CHL. Keywords: Common variable immune deficiency, conductive hearing loss, sensorineural hearing loss

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López-Aldabe, K., L. Hidalgo, A. Antolí, G. Rocamora, X. Corbella, and X. Solanich. "OP0004 AUTOIMMUNE AND INFLAMMATORY MANIFESTATIONS IN COMMON VARIABLE IMMUNODEFICIENCY DISORDERS." Annals of the Rheumatic Diseases 80, Suppl 1 (May 19, 2021): 1.2–2. http://dx.doi.org/10.1136/annrheumdis-2021-eular.2696.

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Background:Common variable immunodeficiency (CVID) disorders are the second most frequent immunodeficiency worldwide and autoimmune diseases (AD) are present in 20% of such patients, cytopenia being the most frequent manifestation [1]. Defects in central and peripheral tolerance, activation/proliferation of B cells, and hypogammaglobulinemia are key features of the disease, along with a reduction in CD4+T cells, abnormalities in Treg and defective secretion of regulatory cytokines, that could perpetuate autoimmune - autoinflammatory phenomena.Objectives:To describe immune and inflammatory disorders in our CVID cohort.Methods:Retrospective analysis of 33 patients who fulfill the European Society for Immunodeficiencies (ESID) Registry – Work criteria for CVID diagnosis [2] treated in the immunodeficiency unit of our tertiary university hospital. After getting an informed consent form, medical records were revised to obtain clinical, analytical and immunological data.Results:Of the 33 CVID patients analysed, 11 had some autoimmune/inflammatory manifestation. Seven patients presented autoimmune thrombocytopenia (AIT), and one of them also had non-severe neutropenia. Two patients also had seronegative spondyloarthropathy, one patient had cutaneous psoriasis, and two patients had alopecia. There were no cases of type 1 diabetes, vitiligo or thyroid disorders. Interestingly, four patients had lung involvement, two of them with granulomatous-lymphocytic interstitial lung disease (GLILD), one with organising pneumonia and one with usual interstitial pneumonia. Five of the eleven patients required immunosuppressive treatment, mostly with steroids. One case of AIT required concomitant treatment with azathioprine and the patient with psoriasis was treated with methotrexate. In six patients, autoimmune disorder was the first manifestation.Conclusion:33% of the CVID patients had autoimmune or inflammatory manifestations in our cohort; six patients had one immune/inflammatory phenomena, four had two different disorders and one patient presented with three different ones. The most frequent manifestation was AIT, as seen in previous reports. In six cases, the autoimmune-inflammatory manifestation was the first symptom attributable to CVID. In conclusion, AD are common in CVID patients, so clinicians must be aware of possible immunodeficiencies in this type of patients.References:[1]Amaya-Uribe L, Rojas M, Azizi G, Anaya JM, Gershwin ME. Primary immunodeficiency and autoimmunity: A comprehensive review. J Autoimmun. 2019 May;99:52-72.[2]ESID Registry – Working Definitions for Clinical Diagnosis of PID, January 22, 2019.Disclosure of Interests:None declared

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Li, Ran, Yali Zheng, Yuqian Li, Rongbao Zhang, Fang Wang, Donghong Yang, Yanliang Ma, Xinlin Mu, Zhaolong Cao, and Zhancheng Gao. "Common Variable Immunodeficiency with Genetic Defects Identified by Whole Exome Sequencing." BioMed Research International 2018 (September 30, 2018): 1–7. http://dx.doi.org/10.1155/2018/3724630.

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Common variable immunodeficiency (CVID) belongs to the primary immunodeficiency disorders (PIDs), presenting a profound heterogeneity in phenotype and genotype, with monogenic or complex causes. Recurrent respiratory infections are the most common clinical manifestations. CVID patients can also develop various autoimmune and lymphoproliferative complications. Genetic testing such as whole exome sequencing (WES) can be utilized to investigate likely genetic defects, helping for better clinical management. We described the clinical phenotypes of three sporadic cases of CVID, who developed recurrent respiratory infections with different autoimmune and lymphoproliferative complications. WES was applied to screen disease-causing or disease-associated mutations. Two patients were identified to have monogenic disorders, with compound heterozygous mutations in LRBA for one patient and a frameshift insertion in NFKB1 for another. The third patient was identified to be a complex form of CVID. Two novel mutations were identified, respectively, in LRBA and NFKB1. A combination of clinical and genetic diagnosis can be more extensively utilized in the clinical practice due to the complexity and heterogeneity of CVID.

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Cunningham-Rundles, Charlotte. "The many faces of common variable immunodeficiency." Hematology 2012, no. 1 (December 8, 2012): 301–5. http://dx.doi.org/10.1182/asheducation.v2012.1.301.3798316.

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Abstract Common variable immunodeficiency (CVID) is a rare immune deficiency characterized by low levels of serum IgG, IgA, and/or IgM, with a loss of Ab production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and much older adults can be found to have this immune defect. The range of clinical manifestations is broad, including acute and chronic infections, inflammatory and autoimmune diseases, and an increased incidence of cancer and lymphoma. For all of these reasons, the disease phenotype is both heterogeneous and complex. In the past few years, data from large patient registries have revealed that both selected laboratory markers and clinical phenotyping may aid in separating groups of subjects into biologically relevant categories. CVID consists of 2 phenotypes, 1 in which infections are the characteristic and another in which impressive inflammatory and/or hematologic complications also develop, including lymphadenopathy, splenomegaly, autoimmune cytopenias, enteropathy, and/or and granulomatous disease. These phenotypes appear to be stable, are related to immunologic and inflammatory markers, and are predictive of outcomes. This review outlines current understanding about this syndrome based on studies of large cohorts, highlighting the evaluation and treatment of complications and, in particular, the autoimmune and inflammatory conditions that affect these patients.

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Sobko, E. A., I. V. Demko, I. A. Soloveva, A. Yu Kraposhina, N. V. Gordeeva, D. A. Anikin, N. S. Pronkina, and O. P. Ischenko. "Common variable immunodeficiency disorder: a clinical case." Medical Immunology (Russia) 23, no. 1 (March 1, 2021): 185–90. http://dx.doi.org/10.15789/1563-0625-cvi-2089.

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Primary immunodeficiency is a rare congenital pathology associated with failure of immune system, manifested by disturbances of its functions. These defects lead to increased susceptibility of patients to various infectious agents, as well as the development of autoimmune, malignant and other diseases. Primary immunodeficiency is classified as a rare disease, which was previously associated with a poor prognosis with a high risk of mortality in childhood. To date, the emergence of highly effective treatment methods has changed the course and prognosis of these diseases. Clinicians of various specialties increasingly meet with this pathology in everyday practice, including adult age cohorts. In this regard, early diagnosis of primary immunodeficiency in adults becomes relevant, being associated with choosing optimal therapy, prevention of severe internal organ damage, determination of management strategy for the patient, as well as the need to identify inherited disorders and provide information to the patient’s family. Delayed verification of the diagnosis may cause disability of the patient and development of irreversible, often fatal complications. This article presents our own clinical case with a newly diagnosed clinical condition: Common variable immunodeficiency disorder (CVID), the most common form of primary immunodeficiency in adults. The symptoms of common variable immunodeficiency disorder appear in these patients in adulthood, but a high-quality collected history of the disease will allow you to trace symptoms in the patients even since early childhood. There is a common gap for several years between the onset of the disease and clinical diagnosis, since erroneous diagnosis is often made due to non-specific clinical symptoms that resemble other, more frequent diseases. The prognosis of patients with CVID depends on several factors: frequency of infections, structural disorders in the lungs, the occurrence of autoimmune diseases and the success of infection prevention. Thus, a variety of clinical forms of primary immunodeficiency, lack of awareness of doctors about this pathology, complexity of immunological examination in the general medical network lead to the fact that CVID is not diagnosed for long terms, and patients do not receive the necessary pathogenetic therapy. There is a need for drawing attention of doctors of various disciplines to the fact that the recurrent inflammatory processes of various localization, which are difficult to respond to adequate traditional therapy, may be caused by changes in the immune system, including congenital, genetically determined immunodeficiency.

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Mantzaris, Gerassimos J., Despina Chrysovergi, Kalliopi Petraki, Dimitra Rontogianni, Emmanuel Archavlis, Chryssa Papasteriadi, Ioanna Economidou, and George Triantafyllou. "H. pylori infection in common variable immunodeficiency disease (CVID)." Gastroenterology 118, no. 4 (April 2000): A1270. http://dx.doi.org/10.1016/s0016-5085(00)80927-0.

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Gause, Angela, Daniela Christine Inderrieden, Rudolf Laas, Andreas-Christoph Arlt, and Wolfgang Ludwig Gross. "Common Variable Immunodeficiency (CVID) and Inclusion Body Myositis (IBM)." Immunobiology 202, no. 2 (August 2000): 199–203. http://dx.doi.org/10.1016/s0171-2985(00)80067-4.

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Hammarström, L., I. Vorechovsky, and D. Webster. "Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID)." Clinical & Experimental Immunology 120, no. 2 (May 2000): 225–31. http://dx.doi.org/10.1046/j.1365-2249.2000.01131.x.

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Sharma, T., and M. Riedl. "P243 Gastrointestinal manifestations of common variable immunodeficiency disorder (CVID)." Annals of Allergy, Asthma & Immunology 117, no. 5 (November 2016): S94. http://dx.doi.org/10.1016/j.anai.2016.09.255.

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Jacob, C. M. A., A. P. M. Castro, A. C. Pastorino, E. Ciccone, A. M. Lima, L. V. Rizzo, G. A. Corradi, and E. M. G. Carnide. "Common variable immunodeficiency (CVID) and cryptosporidiosis of difficult control." Journal of Allergy and Clinical Immunology 115, no. 2 (February 2005): S154. http://dx.doi.org/10.1016/j.jaci.2004.12.628.

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Goldacker, S., R. Draeger, K. Warnatz, D. Huzly, U. Salzer, J. Thiel, H. Eibel, M. Schlesier, and H. H. Peter. "Active vaccination in patients with common variable immunodeficiency (CVID)." Clinical Immunology 124, no. 3 (September 2007): 294–303. http://dx.doi.org/10.1016/j.clim.2007.04.011.

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Jeong, D., S. Skoda-Smith, A. Shimamura, and H. Ochs. "Dyskeratosis Congenita (DC) Presenting as Common Variable Immunodeficiency (CVID)." Journal of Allergy and Clinical Immunology 125, no. 2 (February 2010): AB14. http://dx.doi.org/10.1016/j.jaci.2009.12.086.

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Long, Christopher P., Hiroshi Suzuki, and Kenneth Vitale. "Peripheral Neuropathy Due to Common Variable Immunodeficiency: Case Report and Narrative Review." Gerontology and Geriatric Medicine 5 (January 2019): 233372141985064. http://dx.doi.org/10.1177/2333721419850644.

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A 63-year-old woman with common variable immunodeficiency (CVID) presented with 1 year of insidious onset lower extremity pain and weakness. She underwent a circuitous workup, failed to improve despite treatment for various presumed diagnoses. She presented to a University physical medicine and rehabilitation clinic with continued symptoms. Electrophysiologic testing was recommended revealing a lower extremity motor greater than sensory axonal neuropathy. While CVID has known central nervous system complications, to our knowledge, this represents the second known reported case of peripheral neuropathy. We review the literature on CVID and summarize neurological disease mechanisms and manifestations. Although peripheral neuropathy is a rarely documented complication of CVID, providers need to be aware of potential peripheral nervous system complications of primary immune deficiencies such as CVID due to its significant impact on physical performance, balance, and fall risks.

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Karaulov, Alexander V., Irina V. Sidorenko, and Anna S. Kapustina. "Major approaches in early diagnostics of common variable immunodeficiency in adults in Moscow." F1000Research 1 (November 9, 2012): 46. http://dx.doi.org/10.12688/f1000research.1-46.v1.

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Common variable immunodeficiency (CVID) is a primary immunological disease characterized predominantly by hypogammaglobulinemia. The main clinical manifestations are severe recurrent infections that often lead to structural damage of affected organs. The early start of adequate intravenous immunoglobulin therapy has significantly improved the prognosis of this serious disorder. Patients with CVID are also predisposed to autoimmune and lymphoproliferative complications. This article deals with the features of this primary immunodeficiency in adults. Clinical manifestations, immunological features and treatment concepts were gathered during 21 years of observation of such patients in Moscow. The authors suggest early predictive clinical signs of CVID in adults.

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Khan, Rishad, Mohamad Habbal, Michael A. Scaffidi, Abbas A. Bukhari, Amir Rumman, Sarah Al Ghamdi, Stephen D. Betschel, and Samir C. Grover. "Gastrointestinal Disease in Patients with Common Variable Immunodeficiency: A Retrospective Observational Study." Journal of the Canadian Association of Gastroenterology 3, no. 4 (February 25, 2019): 162–68. http://dx.doi.org/10.1093/jcag/gwz004.

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Abstract Background Gastrointestinal (GI) symptoms are common among patients with common variable immunodeficiency disorder (CVID) yet remain poorly understood. Aims The aim of this study was to characterize the demographic, clinical, endoscopic and histologic features of patients with CVID and GI symptoms. Methods We conducted a retrospective observational study of all patients with CVID at a large Canadian tertiary care centre between January 2000 and May 2018. Results We included 95 patients with CVID. The mean age of patients at the time of CVID diagnosis was 38.2(±16.0). Fifty-three (56%) patients were female. Sixty-four (67%) patients had GI symptoms, with a mean age of onset for GI symptoms of 43.4(±15.1) years. The most common symptoms were bowel movement changes (n = 55 [58%]) and abdominal pain (n = 44 [46%]). Patients with GI symptoms were more likely to have anemia (n = 23 [36%] versus n = 3 [10%], P = 0.0129), iron deficiency (n = 16 [25%] versus n = 2 [7%], P = 0.0481), and have received GI antibiotics (n = 37 [58%] versus n = 0, P < 0.0001) and proton pump inhibitors for reflux (n = 24 [38%] versus n = 3 [10%], P = 0.0067). The most common GI infections were Giardia lamblia (n = 14 [15%]) and Clostridium difficile (n = 4 [4%]). Forty-three (45%) patients with GI symptoms underwent colonoscopy, esophagogastroduodenoscopy or both. The most common findings were inflammation, nodular lymphoid hyperplasia, reduced plasma cells and increased intraepithelial lymphocytes. Conclusions This is the largest study on CVID patients in a North American setting. The majority of patients experienced GI symptoms. Future studies should study response to treatment for GI disease among patients with CVID.

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Baloh, Carolyn H., Michele R. Henson, Anupama Reddy, Rebecca H. Buckley, and Patricia L. Lugar. "A study of cancer incidence in the most common immunodeficiency, common variable immunodeficiency (CVID)." Journal of Allergy and Clinical Immunology 143, no. 2 (February 2019): AB79. http://dx.doi.org/10.1016/j.jaci.2018.12.245.

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Wehr, Claudia, Teemu Kivioja, Christian Schmitt, Berne Ferry, Torsten Witte, Efrem Eren, Marcela Vlkova, et al. "The EUROclass trial: defining subgroups in common variable immunodeficiency." Blood 111, no. 1 (January 1, 2008): 77–85. http://dx.doi.org/10.1182/blood-2007-06-091744.

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The heterogeneity of common variable immunodeficiency (CVID) calls for a classification addressing pathogenic mechanisms as well as clinical relevance. This European multicenter trial was initiated to develop a consensus of 2 existing classification schemes based on flowcytometric B-cell phenotyping and the clinical course. The clinical evaluation of 303 patients with the established diagnosis of CVID demonstrated a significant coincidence of granulomatous disease, autoimmune cytopenia, and splenomegaly. Phenotyping of B-cell subpopulations confirmed a severe reduction of switched memory B cells in most of the patients that was associated with a higher risk for splenomegaly and granulomatous disease. An expansion of CD21low B cells marked patients with splenomegaly. Lymphadenopathy was significantly linked with transitional B-cell expansion. Based on these findings and pathogenic consideration of B-cell differentiation, we suggest an improved classification for CVID (EUROclass), separating patients with nearly absent B cells (less than 1%), severely reduced switched memory B cells (less than 2%), and expansion of transitional (more than 9%) or CD21low B cells (more than 10%). Whereas the first group contains all patients with severe defects of early B-cell differentiation, severely reduced switched memory B cells indicate a defective germinal center development as found in inducible constimulator (ICOS) or CD40L deficiency. The underlying defects of expanded transitional or CD21low B cells remain to be elucidated. This trial is re-gistered at http://www.uniklinik-freiburg.de/zks/live/uklregister/Oeffentlich.html as UKF000308.

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Alkan, Gülsüm, Sevgi Keles, and İsmail Reisli. "Evaluation of Clinical and Immunological Characteristics of Children with Common Variable Immunodeficiency." International Journal of Pediatrics 2018 (2018): 1–8. http://dx.doi.org/10.1155/2018/3527480.

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Background. Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder (PID) that typically presents with hypogammaglobulinemia and impaired antibody production. Objectives. This study aimed to promote the awareness of CVID, whose clinical spectrum is quite broad. Methods. The demographic, clinical, and laboratory characteristics of 12 children (seven males and five females) with CVID were analyzed retrospectively. The patients were diagnosed using the diagnostic criteria of the European Society for Primary Immunodeficiencies. Results. The median disease onset age was 7.2±4.1 years, and the mean diagnosis age was 11.6±3.7 years. The diagnosis delay was 4.3±2.6 years, and the parental consanguinity rate was 75%. Most patients presented with recurrent infections, including upper respiratory tract infections (n=8), lower respiratory tract infections (n=9), and gastroenteritis (n=5). In addition, growth retardation (n=9) and bronchiectasis (n=5) were common comorbidities. Two patients presented with autoimmune thrombocytopenia and anemia, and one patient exhibited lung empyema. All the patients had immunoglobulin G deficiencies. Conclusion. CVID is a heterogeneous disease, so the diagnosis is frequently delayed. In the CVID patients with pulmonary complications, relationships were seen with the diagnosis delay, symptom onset age, and lung infection prevalence. Overall, the early diagnosis and treatment of PIDs can preclude life-threatening complications.

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Wheat, William H., Carlyne D. Cool, Yoshikazu Morimoto, Pradeep R. Rai, Charles H. Kirkpatrick, Barbara A. Lindenbaum, Christopher A. Bates, et al. "Possible role of human herpesvirus 8 in the lymphoproliferative disorders in common variable immunodeficiency." Journal of Experimental Medicine 202, no. 4 (August 15, 2005): 479–84. http://dx.doi.org/10.1084/jem.20050381.

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Patients who have common variable immunodeficiency (CVID) and granulomatous/lymphocytic interstitial lung disease (GLILD) are at high risk for early mortality and B cell lymphomas. Infection with human herpes virus type 8 (HHV8), a B cell lymphotrophic virus, is linked to lymphoproliferative disorders in people who have secondary immunodeficiencies. Therefore, we determined the prevalence of HHV8 infection in CVID patients with GLILD. Genomic DNA isolated from peripheral blood mononuclear cells was screened by nested- and real time-quantitative PCR (QRT-PCR) for the presence of HHV8 genome. It was positive in 6/9 CVID patients with GLILD (CVID-GLILD), 1/21 CVID patients without GLILD (CVID-control), and no patients receiving intravenous gamma globulin (n = 13) or normal blood donors (n = 20). Immunohistochemistry (IHC) demonstrated expression of the latency-associated nuclear antigen-1 (LANA-1) in the biopsies of the lung, liver, and bone marrow of four patients with CVID-GLILD. One CVID-GLILD patient developed a B cell lymphoma during the course of the study. QRT-PCR demonstrated high copy number of HHV8 genome and IHC showed diffuse staining for LANA-1 in the malignant lymph node. HHV8 infection may be an important factor in the pathogenesis of the interstitial lung disease and lymphoproliferative disorders in patients with CVID.

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Gullo, Irene, Catarina Costa, Susana L. Silva, Cristina Ferreira, Adriana Motta, Sara P. Silva, Rúben Duarte Ferreira, et al. "The Dysfunctional Immune System in Common Variable Immunodeficiency Increases the Susceptibility to Gastric Cancer." Cells 9, no. 6 (June 19, 2020): 1498. http://dx.doi.org/10.3390/cells9061498.

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Gastric carcinoma (GC) represents the most common cause of death in patients with common variable immunodeficiency (CVID). However, a limited number of cases have been characterised so far. In this study, we analysed the clinical features, bacterial/viral infections, detailed morphology and immune microenvironment of nine CVID patients with GC. The study of the immune microenvironment included automated digital counts of CD20+, CD4+, CD8+, FOXP3+, GATA3+ and CD138+ immune cells, as well as the evaluation of PD-L1 expression. Twenty-one GCs from non-CVID patients were used as a control group. GC in CVID patients was diagnosed mostly at early-stage (n = 6/9; 66.7%) and at younger age (median-age: 43y), when compared to non-CVID patients (p < 0.001). GC pathogenesis was closely related to Helicobacter pylori infection (n = 8/9; 88.9%), but not to Epstein-Barr virus (0.0%) or cytomegalovirus infection (0.0%). Non-neoplastic mucosa (non-NM) in CVID-patients displayed prominent lymphocytic gastritis (100%) and a dysfunctional immune microenvironment, characterised by higher rates of CD4+/CD8+/Foxp3+/GATA3+/PD-L1+ immune cells and the expected paucity of CD20+ B-lymphocytes and CD138+ plasma cells, when compared to non-CVID patients (p < 0.05). Changes in the immune microenvironment between non-NM and GC were not equivalent in CVID and non-CVID patients, reflecting the relevance of immune dysfunction for gastric carcinogenesis and GC progression in the CVID population.

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Amer, R., G. Bamonte, and J. V. Forrester. "Resolution of Juvenile Idiopathic Arthritis-Associated Uveitis after Development of Common Variable Immunodeficiency." European Journal of Ophthalmology 17, no. 4 (July 2007): 666–68. http://dx.doi.org/10.1177/112067210701700429.

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Purpose To describe the occurrence of common variable immunodeficiency (CVID) in a patient with juvenile idiopathic arthritis (JIA) and JIA-associated uveitis. Methods/Results Case report. A 29-year-old woman was followed-up since the age of 10 years because of right eye JIA-associated recurrent anterior uveitis. She was treated with steroids and immunosuppressants with good control of uveitis and arthritis. At the age of 17 years, she did not experience any further relapse of uveitis or arthritis and both diseases were considered to be in remission. Concomitantly, she started to have recurrent infections and later she underwent splenectomy because of autoimmune hemolytic anemia and thrombocytopenia. Liver biopsy disclosed granulomatous hepatitis. She was ultimately diagnosed with CVID at the age of 23 years when her blood tests revealed neutropenia and severe panhypogammaglobulinemia. She has been treated since then with intravenous immunoglobulins with good control of the disease. Since the development of CVID, she has had no relapses of uveitis or arthritis during a follow-up period of 12 years. Conclusions Common variable immunodeficiency (CVID) is the most common primary immunodeficiency where defective antibody formation is the most common feature with B-cell differentiation failure. Ocular complications have been rarely documented and included bacterial conjunctivitis, retinal vasculitis and multifocal choroiditis. We herein report on the occurrence of JIA-associated uveitis as a comorbid manifestation of CVID. We speculate a role for B cells in the pathogenesis of JIA and JIA-associated uveitis here, as this patient had total remission of both conditions with the onset of CVID.

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Journal articles on the topic 'Common variable immunodeficiency (CVID)'

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