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Igg4 immunodeficiency

Jiraprapa Wipasa, Romanee Chaiwarith, Kriangkrai Chawansuntati, Jutarat Praparattanapan, Kritsadee Rattanathammethee, Khuanchai Supparatpinyo
A major characteristic of immunodeficiency associated with life-threatening intracellular infection in adults is the presence of anti-interferon-γ antibodies. Although little is known about the mechanism underlying this syndrome, it is believed that the antibodies inhibit the activity of downstream signaling pathway of interferon-γ. In this study, the characteristics of these antibodies in patients who presented, or have a history of, intracellular infection and were positive to anti-interferon-γ antibodies were investigated...
January 1, 2018: Experimental Biology and Medicine
Elena Blanco, Martín Pérez-Andrés, Sonia Arriba-Méndez, Teresa Contreras-Sanfeliciano, Ignacio Criado, Ondrej Pelak, Ana Serra-Caetano, Alfonso Romero, Noemí Puig, Ana Remesal, Juan Torres Canizales, Eduardo López-Granados, Tomas Kalina, Ana E Sousa, Menno Van Zelm, Mirjam Van der Burg, Jacques J M van Dongen, Alberto Orfao
BACKGROUND: Humoral immunocompetence develops stepwise throughout life and contributes to individual susceptibility to infection, immunodeficiency, autoimmunity and neoplasia. Immunoglobulin heavy chain (IgH)-isotype serum levels can partly explain such age-related differences, but their relationship with the IgH-isotype distribution within memory B-cell (MBCs) and plasma cell (PCs) compartments remains to be investigated. OBJECTIVE: We studied the age-related distribution of MBCs and PCs expressing different IgH-isotypes, in addition to the immature/transitional and naive B-cell compartments...
March 2, 2018: Journal of Allergy and Clinical Immunology
Prachi Dua, Roman Shinder, Derek B Laskar, Douglas R Lazzaro, Allison E Rizzuti
Purpose: To report a case of hypertrophic herpes simplex virus (HSV) of the eyelid and cornea masquerading as IgG4-related disease. Observations: A 37-year old African American female with a past medical history of human immunodeficiency virus (HIV) on highly active antiretroviral therapy (HAART) and a recent history of treated genital herpes, presented with an ulcerative lesion of the left upper and lower eyelids, and severe ocular inflammation with symblepharon...
March 2018: American Journal of Ophthalmology Case Reports
Monica G Lawrence, Thamiris V Palacios-Kibler, Lisa J Workman, Alexander J Schuyler, John W Steinke, Spencer C Payne, Emily C McGowan, James Patrie, Ramsay L Fuleihan, Kathleen E Sullivan, Patricia L Lugar, Camellia L Hernandez, Douglas E Beakes, James W Verbsky, Thomas A E Platts-Mills, Charlotte Cunningham-Rundles, John M Routes, Larry Borish
Although small prior studies have suggested that IgE can be low in common variable immunodeficiency (CVID), the workup for patients with recurrent infections and suspected hypogammaglobulinemia does not include the routine measurement of serum IgE. We sought to test the hypothesis that low/undetectable serum IgE is characteristic of CVID by comparing the frequency of low/undetectable serum IgE in healthy controls and patients with CVID. We measured total serum IgE in a large multi-center cohort of patients with CVID (n = 354) and compared this to large population-based cohorts of children and adults...
February 17, 2018: Journal of Clinical Immunology
Grace van der Gugten, Mari L DeMarco, Luke Y C Chen, Alex Chin, Mollie Caruthers, Daniel T Holmes, Andre Mattman
BACKGROUND: The Binding Site immunonephelometric (IN) IgG subclass reagents (IgG1, IgG2, IgG3, IgG4, BSIN) are used for assessment of both immunodeficiency and IgG4-related disease (IgG4-RD). In our laboratory, suspected analytic errors were noted in patients with increases in IgG4: The sum of the individual IgG subclasses was substantially greater than the measured total IgG concentrations (unlike samples with normal IgG4), and the IgG4 concentration was always less than the IgG2 concentration...
January 19, 2018: Clinical Chemistry
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
Evan S Marlin, David Dornbos, Daniel S Ikeda, Norman L Lehman, Ciarán J Powers
BACKGROUND: Diffuse intracranial aneurysmal vasculopathy is a rare condition, previously described in patients with human immunodeficiency virus infection. IgG4-related disease (IgG4-RD) is a recognized inflammatory disease of systemic organs, leading to fibrosis of connective tissues. It also has been linked to inflammatory dilating aortic aneurysms, coronary vascular disease, hypophysitis, orbital pseudotumor, and pachymeningitis. It has not yet been described as a cause of diffuse intracranial dilating vasculopathy...
November 2017: World Neurosurgery
Hsing-Tse Yu, Chen-Hsiang Lee, Shun-Chen Huang, Shan-Fu Yu
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition characterized by infiltration of the involved organs by IgG4-bearing plasma cells. The prevalence of autoimmune diseases, associated with or occurring in patients with human immunodeficiency virus (HIV) infection, has been increasing. We describe a 58-year-old man with an undiagnosed HIV infection, which presented as chronic cervical lymphadenopathy with an elevated serum IgG4 and a very high IgE. Histologically, lymph nodes showed expanded sinusoids and burnt-out germinal centers with increased plasmacytic infiltration and collagen fiber deposition...
January 2018: International Journal of STD & AIDS
Zita Chovancova, Pavlina Kralickova, Alena Pejchalova, Marketa Bloomfield, Jana Nechvatalova, Marcela Vlkova, Jiri Litzman
PURPOSE: Primary selective IgM deficiency (sIgMD) is a primary immunodeficiency with unclear pathogenesis and a low number of published cases. METHODS: We reviewed clinical and laboratory manifestations of 17 sIgMD patients. Serum IgM, IgG, and its subclasses, IgA, IgE, antibodies against tetanus toxoid, pneumococcal polysaccharides and Haemophilus influenzae type b, isohemagglutinins, and T and B lymphocyte subsets, expressions of IgM on B cells and B lymphocyte production of IgM were compared with previously reported case reports and a small series of patients, which included 81 subjects in total...
August 2017: Journal of Clinical Immunology
Sven Kratochvil, Paul F McKay, Jakub T Kopycinski, Cynthia Bishop, Peter John Hayes, Luke Muir, Christopher L Pinder, Deniz Cizmeci, Deborah King, Yoann Aldon, Bruce D Wines, P Mark Hogarth, Amy W Chung, Stephen J Kent, Kathrin Held, Christof Geldmacher, Len Dally, Nelson S Santos, Tom Cole, Jill Gilmour, Sarah Fidler, Robin J Shattock
A key aspect to finding an efficacious human immunodeficiency virus (HIV) vaccine is the optimization of vaccine schedules that can mediate the efficient maturation of protective immune responses. In the present study, we investigated the effect of alternate booster regimens on the immune responses to a candidate HIV-1 clade C CN54gp140 envelope protein, which was coadministered with the TLR4-agonist glucopyranosyl lipid A-aqueous formulation. Twelve study participants received a common three-dose intramuscular priming series followed by a final booster at either 6 or 12 months...
2017: Frontiers in Immunology
S Sanges, F Wallet, N Blondiaux, D Theis, I Vérin, A Vachée, R Dessein, K Faure, N Viget, E Senneville, O Leroy, F Maury, N Just, J Poissy, D Mathieu, A Prévotat, C Chenivesse, A Scherpereel, G Smith, B Lopez, J Rosain, V Frémeaux-Bacchi, E Hachulla, P-Y Hatron, M Bahuaud, F Batteux, D Launay, M Labalette, G Lefèvre
OBJECTIVES: Screening for primary immunodeficiencies (PIDs) in adults is recommended after two severe bacterial infections. We aimed to evaluate if screening should be performed after the first invasive infection in young adults. METHODS: Eligible patients were retrospectively identified using hospital discharge and bacteriology databases in three centres during a 3-year period. Eighteen to 40-year-old patients were included if they had experienced an invasive infection with encapsulated bacteria commonly encountered in PIDs (Streptococcus pneumoniae (SP), Neisseria meningitidis (NM), Neisseria gonorrhoeae (NG), Haemophilus influenzae (HI), or group A Streptococcus (GAS))...
August 2017: Clinical Microbiology and Infection
M Elkuch, V Greiff, C T Berger, M Bouchenaki, T Daikeler, A Bircher, A A Navarini, I Heijnen, M Recher
During the last two decades, hyper-immunoglobulin (Ig)E syndromes have been characterized clinically and molecularly in patients with genetically determined primary immunodeficiencies. However, the detection of low IgE levels, defined here as below detection limit in the routine clinical immunology laboratory, has received little attention. We analysed the association of serum IgA, IgM and IgG levels (including IgG subclasses) with low, normal or high serum IgE levels in patients evaluated in a single-centre out-patient immunodeficiency and allergy clinic...
March 2017: Clinical and Experimental Immunology
Štefan Blazina, Alojz Ihan, Luca Lovrečić, Tinka Hovnik
Antibody deficiency is common finding in patients with Jacobsen syndrome (JS). In addition, there have been few reports of T-cell defects in this condition, possibly because most of the reported patients have not been specifically evaluated for T-cell function. In this article, we present a child with an 11q deletion and combined immunodeficiency and we perform a literature overview on immunodeficiency in JS. Our patient presented with recurrent bacterial and prolonged viral infections involving the respiratory system, as well as other classic features of the syndrome...
December 2016: American Journal of Medical Genetics. Part A
Joo Hee Kim, Sunghoon Park, Yong Il Hwang, Seung Hun Jang, Ki Suck Jung, Yun Su Sim, Cheol Hong Kim, Changhwan Kim, Dong Gyu Kim
Immunoglobulin G subclass deficiency (IgGSCD) is a relatively common primary immunodeficiency disease (PI) in adults. The biological significance of IgGSCD in patients with chronic airway diseases is controversial. We conducted a retrospective study to characterize the clinical features of IgGSCD in this population. This study examined the medical charts from 59 adult patients with IgGSCD who had bronchial asthma or chronic obstructive pulmonary disease (COPD) from January 2007 to December 2012. Subjects were classified according to the 10 warning signs developed by the Jeffrey Modell Foundation (JMF) and divided into two patient groups: group I (n = 17) met ≥ two JMF criteria, whereas group II (n = 42) met none...
October 2016: Journal of Korean Medical Science
Aleksandra Szczawinska-Poplonyk, Irena Wojsyk-Banaszak, Katarzyna Jonczyk-Potoczna, Anna Breborowicz
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a multiorgan fibroinflammatory condition with lymphoplasmacytic infiltrates containing abundant IgG4-positive plasma cells. The immunopathogenesis of the disease and the potential role of triggering autoantigens or infectious factors have not been clearly defined. Immunoglobulin G4-related lung disease is a new and emerging condition in pediatric patients and to date, there have been only two reports regarding pulmonary manifestation of IgG4-RD in children recently published...
June 8, 2016: Italian Journal of Pediatrics
Sandro F Perazzio, Átila Granados, Reinaldo Salomão, Neusa P Silva, Magda Carneiro-Sampaio, Luis Eduardo C Andrade
OBJECTIVE: To determine the frequency of immunodeficiency-like states in SLE and related clinical features. METHODS: Three hundred and fifteen SLE patients and 301 controls were evaluated for C4A and C4B gene copy number, immunoglobulin isotypes, IgG subclasses, total haemolytic complement (CH50), C2, C3 and neutrophil oxidative burst. C2 and C3 genes were sequenced in cases of low C2 or C3 levels. Those presenting abnormal CH50 with normal C2 and C3 underwent C1q-C9 determination...
September 2016: Rheumatology
Haitham Odat, Mohannad Alqudah
The purpose of this study is to readdress the issue of primary humoral immunodeficiency frequency and pattern in medically resistant chronic rhinosinusitis (CRS) based on the new guidelines for CRS diagnosis and management. Two hundred and fifty-seven consecutive patients with refractory CRS were included in this study. The results of their IgA, IgM, IgG, and IgG subclasses were analyzed and compared with 75 age- and gender-matched control groups. The average age of CRS patients was 34 years (SD ± 13). In the refractory CRS group, there was no significant difference between patients with or without humoral immunodeficiency based on age, gender and status of allergy, polyps and revision...
October 2016: European Archives of Oto-rhino-laryngology
Katariina Hannula-Jouppi, Satu-Leena Laasanen, Mette Ilander, Laetitia Furio, Mirja Tuomiranta, Riitta Marttila, Leila Jeskanen, Valtteri Häyry, Mervi Kanerva, Sirpa Kivirikko, Marja-Leena Tuomi, Hannele Heikkilä, Satu Mustjoki, Alain Hovnanian, Annamari Ranki
IMPORTANCE: Netherton syndrome (NS) is a rare and severe genodermatosis caused by SPINK5 mutations leading to the loss of lymphoepithelial Kazal-type-related inhibitor (LEKTI). Netherton syndrome is characterized by neonatal scaling erythroderma, a bamboolike hair defect, a substantial skin barrier defect, and a profound atopic diathesis. Netherton syndrome has been proposed to be a primary immunodeficiency syndrome because of the high frequency of infections. The precise mechanisms underlying the disease are not fully understood...
April 2016: JAMA Dermatology
Ting-Ting Liu, Shao-Wen Weng, Ming-Chung Wang, Wan-Ting Huang
Disseminated nontuberculous mycobacteria (NTM) infection with concurrent IgG4-related lymphadenopathy has not been reported. We described a patient with neutralizing autoantibodies to interferon-gamma (IFN-γ) and elevated levels of serum IgG4 presenting with generalized lymphadenopathy and reactive dermatosis. Histologically, lymph nodes (LNs) showed effaced nodal architecture with polymorphic infiltrates, mimicking angioimmunoblastic T-cell lymphoma. Both the absolute number and the ratio of IgG4+ plasma cells to IgG+ plasma cells were increased...
March 2016: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
Francesco Rapisarda, Luca Zanoli, Grazia Portale, Salvo Scuto, Pietro Castellino
The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm(3) (or <20% of total T cells) in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells...
2015: Case Reports in Medicine
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