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IgG4 subclass deficiency

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
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
Caroline Pfeiffer, Eve Mathieu-Dupas, Pauline Logghe, Géraldine Lissalde-Lavigne, Julien Balicchi, Umran Caliskan, Thomas Valentin, Daniel Laune, Franck Molina, Jean François Schved, Muriel Giansily-Blaizot
While the immune response to hemophilic factors in hemophilia has been widely studied, little is known about the development of anti-Factor VII (FVII) antibodies in FVII deficiency. We developed a robust technique based on the x-MAP technology to detect the presence of antibodies against FVII and characterize their isotype and validated this method using blood samples from 100 patients with FVII deficiency (median FVII clotting activity [FVII:C]: 6%) and 95 healthy controls. Anti-FVII antibodies were detected in patients but also in some controls, although the concentration of total immunoglobulin G (IgGt) and IgG1 and IgG4 subclasses was significantly different between groups...
May 2016: Thrombosis Research
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
Fernanda S Nascimento, Lisandra A Suzuki, Nilson Branco, Regina M B Franco, Paula D Andrade, Sandra C B Costa, Marcelo N Pedro, Cláudio L Rossi
Cerebral toxoplasmosis can be highly debilitating and occasionally fatal in persons with immune system deficiencies. In this study, we evaluated the Toxoplasma gondii-specific IgG subclass antibody response in 19 cerebrospinal fluid (CSF) samples from patients with cerebral toxoplasmosis who had a positive IgG anti-T. gondii ELISA standardized with a cyst antigen preparation. There were no significant differences between the rates of positivity and the antibody concentrations (arithmetic means of the ELISA absorbances, MEA) for IgG1 and IgG2, but the rates of positivity and MEA values for these two IgG subclasses were significantly higher than those for IgG3 and IgG4...
September 2015: Revista do Instituto de Medicina Tropical de São Paulo
Shahin Gaini
A 51-year-old Caucasian woman with Listeria monocytogenes meningitis was treated and discharged after an uncomplicated course. Her medical history included immunosuppressive treatment with prednisolone and azathioprine for autoimmune hepatitis. A diagnostic work-up after the meningitis episode revealed that she had low levels of the IgG4 subclass. To our knowledge, this is the first case report describing a possible association between autoimmune hepatitis and the occurrence of Listeria monocytogenes meningitis, describing a possible association between Listeria monocytogenes meningitis and deficiency of the IgG4 subclass and finally describing a possible association between Listeria monocytogenes meningitis and immunosuppressive therapy with prednisolone and azathioprine...
2015: Case Reports in Infectious Diseases
G Manfredi, L Dell'Aera, R Liguori
PROBLEM: It's well known that iv. immunoglobulins may be useful to overcome habitual abortions, but the mechanisms at the base of a successful outcome and the likelihoods are still unknown. METHOD OF STUDY: In one hundred and sixty women with habitual abortions and one hundred and sixty healthy mothers, we evaluated blood IgG subclasses; among the patients, sixteen merely showed IgG subclass deficiency, after leaving out any autoimmunity and/or coagulation disorders...
May 2015: European Annals of Allergy and Clinical Immunology
Leif G Hanitsch, Madlen Löbel, Holger Müller-Redetzky, Mariana Schürmann, Norbert Suttorp, Nadine Unterwalder, Ulrike Mönnich, Christian Meisel, Kirsten Wittke, Hans-Dieter Volk, Carmen Scheibenbogen, Uwe Kölsch
PURPOSE: Since we described for the first time a patient with IgG4 autoantibodies to IFN-γ more than 10 years ago, many patients with IFN-γ IgG4 autoantibodies have been described, mostly in Mongolian/ Asian patients with a particular HLA background and in association with disseminated nontuberculous mycobacterial infections. Very recently, the first Caucasian US patient was reported and we now present the case of a 65-year old Caucasian woman with severe disseminated Mycobacterium avium infection, cerebral toxoplasmosis and salmonella sepsis who was tested positive for IFN-γ deficiency due to unusual anti-IFN-γ IgG1 autoantibodies...
May 2015: Journal of Clinical Immunology
Marina Vivarelli, Francesco Emma, Thimothée Pellé, Christopher Gerken, Stefania Pedicelli, Francesca Diomedi-Camassei, Günter Klaus, Siegfried Waldegger, Pierre Ronco, Hanna Debiec
Alloimmune antenatal membranous nephropathy (MN) during pregnancy results from antibodies produced by a neutral endopeptidase (NEP)-deficient mother. Here we report two recent cases that provide clues to the severity of renal disease. Mothers of the two children had circulating antibodies against NEP showing the characteristic species-dependent pattern by immunofluorescence on kidney slices. A German mother produced predominantly anti-NEP IgG4 accompanied by a low amount of IgG1. Her child recovered renal function within a few weeks...
March 2015: Kidney International
Pirkka T Pekkarinen, Nelli Heikkilä, Kai Kisand, Pärt Peterson, Marina Botto, Mohamed R Daha, Christian Drouet, Lourdes Isaac, Merja Helminen, Tari Haahtela, Seppo Meri, Hanna Jarva, T Petteri Arstila
In addition to its effector functions, complement is an important regulator of adaptive immune responses. Murine studies suggest that complement modulates helper T-cell differentiation, and Th1 responses in particular are impaired in the absence of functional complement. Here, we have studied humoral responses to toxoid vaccines in eight patients with C3 deficiency, representing more than 25% of all the known patients worldwide. Serum cytokine levels were also studied. The patients developed normal Ig responses to tetanus and diphtheria toxoids, but IgE levels were low...
March 2015: European Journal of Immunology
Luigi F Bertoli, Dennis G Pappas, J Clayborn Barton, James C Barton
BACKGROUND: Our informal observations suggested that some patients with acute sensorineural hearing loss (ASNHL) have subnormal serum immunoglobulin (Ig) levels. We evaluated 28 consecutive adults (18 men, 10 women) at ASNHL diagnosis using: antibodies to 68 kD protein, 30 kD protein, and type II collagen; and serum total IgG, IgG subclasses, total IgA, and IgM. Reference ranges for Ig levels were mean ± 2 SD. We compared prevalences of subnormal IgG subclasses to those in 275 healthy European adults in previous reports...
2014: BMC Immunology
James C Barton, Luigi F Bertoli, J Clayborn Barton
Common variable immunodeficiency (CVID) and immunoglobulin (Ig) G subclass deficiency (IgGSD) are heterogeneous disorders characterized by respiratory tract infections, selective Ig isotype deficiencies, and impaired antibody responses to polysaccharide antigens. Using univariable analyses, we compared observations in 34 CVID and 398 IgGSD adult index patients (81.9% women) referred to a hematology/oncology practice. Similarities included specialties of referring physicians, mean ages, proportions of women, reactivity to Pneumovax, median serum IgG3 and IgG4 levels, median blood CD56+/CD16+ lymphocyte levels, positivity for HLA-A and -B types, and frequencies of selected HLA-A, -B haplotypes...
2014: Journal of Immunology Research
Susan J Pizzutto, Stephanie T Yerkovich, John W Upham, Belinda J Hales, Wayne R Thomas, Anne B Chang
Chronic suppurative lung disease (CSLD) is characterized by the presence of a chronic wet or productive cough and recurrent lower respiratory infections. The aim of this study was to identify features of innate, cell-mediated and humoral immunity that may increase susceptibility to respiratory infections in children with CSLD. Because non-typeable Haemophilus influenzae (NTHi) is commonly isolated from the airways in CSLD, we examined immune responses to this organism in 80 age-stratified children with CSLD and compared their responses with 51 healthy control children...
2014: PloS One
Paula M Ladwig, David R Barnidge, Melissa R Snyder, Jerry A Katzmann, David L Murray
BACKGROUND: Measurement of IgG subclasses is a useful tool for investigation of humoral immune deficiency in the presence of total IgG within reference intervals and IgG4-related disease. Nephelometry has been the method of choice for quantification. We describe an LC-MS/MS method that can multiplex all 4 subclasses along with total IgG by use of either IgG subclass-specific peptide stable isotope-labeled internal standards or a surrogate digest standard for quantification and does not rely on antigen/antibody reactions...
August 2014: Clinical Chemistry
Piero Pavone, Raffaele Falsaperla, Kasun De Silva, Rosaria Taibi, Alberto Verrotti, Rosario R Trifiletti, Giovanna Vitaliti
We report four children with Down Syndrome (DS) without evidence of congenital heart disease who sustained cerebral infarction in the context of an infectious disease. In one child, stroke occurred in the context of acute infection with Mycoplasma pneumonia. In another child, stroke occurred in the context of Streptococcus oralis (viridans subgroup) infection. In two other children, stroke occurred in the context of a bibasilar pneumonia for which an etiologic agent was not found. All patients had evidence of selective IgG4 subclass deficiency...
July 2014: European Journal of Paediatric Neurology: EJPN
Raweerat Sitcharungsi, Torsak Bunupuradah, Arree Pornvoranunt, Tanakorn Apornpong, Jintanat Ananworanich, Kalayanee Khupulsup, Phonethipsavanh Nouanthong, Soamarat Vilaiyuk, Chayapa Phasomsap, Wasu Kamchaisatian, Chitsanu Pancharoen, Thanyawee Puthanakit, Chukiat Sirivichayakul, Suwat Benjaponpitak
BACKGROUND: Variation of normal immunoglobulin (Ig) levels between different genetic and environment factors has been studied. Although antibody deficiency diseases can start from infancy, data of Ig reference levels in children aged ≤24 months are still limited, especially in Asian children. PURPOSE: The aim of this study was to determine serum IgG, IgA, IgM, and IgG subclasses in healthy Thai children from the newborn period to age 24 months. METHODS: Serum samples were collected from healthy Thai children age <1-24 months to measured serum IgG, IgA, IgM, and IgG subclasses by nephelometry...
December 2013: Asian Pacific Journal of Allergy and Immunology
Amit Rawat, Deepti Suri, Anju Gupta, Biman Saikia, Ranjana Walker Minz, Surjit Singh
Immunoglobulin G4 subclass deficiency is the most common IgG subclass deficiency, followed by immunoglobulin G2 deficiency. However, IgG2 deficiency is the most common IgG subclass deficiency associated with recurrent infections. Immunoglobulin G4 subclass deficiency occurs in association with other isotype deficiencies. Isolated IgG4 deficiency, however, is much less common. Although respiratory tract infections are the most common manifestation in isolated IgG4 deficiency, other mucosal surfaces may also be affected in IgG4 deficiency leading to diarrhea and recurrent giardiasis...
September 2014: Indian Journal of Pediatrics
Ji Hyeon Baek, Hyun Kyong Seo, Hye Mi Jee, Youn Ho Shin, Man Yong Han, Eun Sang Oh, Hyun Ju Lee, Kyung Hyo Kim
PURPOSE: Although chronic and recurrent rhinosinusitis is prevalent in children, little is known about its causes. Here, we investigated the humoral immunity in children with chronic or recurrent rhinosinusitis. METHODS: We examined 16 children attending the outpatient clinic at the CHA Bundang Medical Center including 11 boys and 5 girls, aged 3.11 years (mean age, 5.6 years), who had rhinosinusitis for >3 months or >3 times per year. The complete blood count with differential and total serum concentrations of Immunoglobulin (Ig) E, IgA, IgD, IgM, IgG, and IgG subclasses (IgG1, IgG2, IgG3, and IgG4) of all children were measured...
July 2013: Korean Journal of Pediatrics
J Kärner, A Meager, M Laan, J Maslovskaja, M Pihlap, A Remm, E Juronen, A S B Wolff, E S Husebye, K T Podkrajšek, N Bratanic, T Battelino, N Willcox, P Peterson, K Kisand
Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is a recessive disorder resulting from mutations in the autoimmune regulator (AIRE). The patients' autoantibodies recognize not only multiple organ-specific targets, but also many type I interferons (IFNs) and most T helper type 17 (Th17) cell-associated cytokines, whose biological actions they neutralize in vitro. These anti-cytokine autoantibodies are highly disease-specific: otherwise, they have been found only in patients with thymomas, tumours of thymic epithelial cells that fail to express AIRE...
March 2013: Clinical and Experimental Immunology
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