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https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#1
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28803167/igg4-related-disease-a-new-etiology-underlying-diffuse-intracranial-dilating-vasculopathy
#2
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
https://www.readbyqxmd.com/read/28768471/unsuspected-human-immunodeficiency-virus-infection-presenting-as-immunoglobulin-g4-related-lymphadenopathy-a-case-report
#3
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 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28730517/selective-igm-deficiency-clinical-and-laboratory-features-of-17-patients-and-a-review-of-the-literature
#4
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...
July 21, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28596770/a-phase-1-human-immunodeficiency-virus-vaccine-trial-for-cross-profiling-the-kinetics-of-serum-and-mucosal-antibody-responses-to-cn54gp140-modulated-by-two-homologous-prime-boost-vaccine-regimens
#5
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
https://www.readbyqxmd.com/read/28192236/diagnosis-of-primary-antibody-and-complement-deficiencies-in-young-adults-after-a-first-invasive-bacterial-infection
#6
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
https://www.readbyqxmd.com/read/28078662/low-immunoglobulin-e-flags-two-distinct-types-of-immune-dysregulation
#7
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
https://www.readbyqxmd.com/read/27605496/11q-terminal-deletion-and-combined-immunodeficiency-jacobsen-syndrome-case-report-and-literature-review-on-immunodeficiency-in-jacobsen-syndrome
#8
REVIEW
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
https://www.readbyqxmd.com/read/27550483/immunoglobulin-g-subclass-deficiencies-in-adult-patients-with-chronic-airway-diseases
#9
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
https://www.readbyqxmd.com/read/27277432/pulmonary-manifestation-of-immunoglobulin-g4-related-disease-in-a-7-year-old-immunodeficient-boy-with-epstein-barr-virus-infection-a-case-report
#10
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
https://www.readbyqxmd.com/read/27274095/high-frequency-of-immunodeficiency-like-states-in-systemic-lupus-erythematosus-a-cross-sectional-study-in-300-consecutive-patients
#11
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
https://www.readbyqxmd.com/read/26975445/prevalence-and-pattern-of-humoral-immunodeficiency-in-chronic-refractory-sinusitis
#12
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
https://www.readbyqxmd.com/read/26865388/intrafamily-and-interfamilial-phenotype-variation-and-immature-immunity-in-patients-with-netherton-syndrome-and-finnish-spink5-founder-mutation
#13
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
https://www.readbyqxmd.com/read/26660641/nontuberculous-mycobacterial-infection-with-concurrent-igg4-related-lymphadenopathy
#14
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
https://www.readbyqxmd.com/read/26491451/a-case-report-of-an-atypical-presentation-of-igg4-related-disease-and-idiopathic-cd4-lymphocytopenia
#15
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
https://www.readbyqxmd.com/read/26308749/comprehensive-analysis-of-the-therapeutic-igg4-antibody-pembrolizumab-hinge-modification-blocks-half-molecule-exchange-in-vitro-and-in-vivo
#16
Xiaoyu Yang, Fengqiang Wang, Ying Zhang, Larry Wang, Svetlana Antonenko, Shuli Zhang, Yi Wei Zhang, Mohammad Tabrizifard, Grigori Ermakov, Derek Wiswell, Maribel Beaumont, Liming Liu, Daisy Richardson, Mohammed Shameem, Alexandre Ambrogelly
IgG4 antibodies are evolving as an important class of cancer immunotherapies. However, human IgG4 can undergo Fab arm (half molecule) exchange with other IgG4 molecules in vivo. The hinge modification by a point mutation (S228P) prevents half molecule exchange of IgG4. However, the experimental confirmation is still expected by regulatory agencies. Here, we report for the first time the extensive analysis of half molecule exchange for a hinge-modified therapeutic IgG4 molecule, pembrolizumab (Keytruda) targeting programmed death 1 (PD1) receptor that was approved for advanced melanoma...
December 2015: Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/26056943/hypermethylation-of-mst1-in-igg4-related-autoimmune-pancreatitis-and-rheumatoid-arthritis
#17
Takataro Fukuhara, Takashi Tomiyama, Kaneki Yasuda, Yoshihiro Ueda, Yoshio Ozaki, Yonsu Son, Shosaku Nomura, Kazushige Uchida, Kazuichi Okazaki, Tatsuo Kinashi
The serine/threonine kinase Mst1 plays important roles in the control of immune cell trafficking, proliferation, and differentiation. Previously, we reported that Mst1 was required for thymocyte selection and regulatory T-cell functions, thereby the prevention of autoimmunity in mice. In humans, MST1 null mutations cause T-cell immunodeficiency and hypergammaglobulinemia with autoantibody production. RASSF5C(RAPL) is an activator of MST1 and it is frequently methylated in some tumors. Herein, we investigated methylation of the promoter regions of MST1 and RASSF5C(RAPL) in leukocytes from patients with IgG4-related autoimmune pancreatitis (AIP) and rheumatoid arthritis (RA)...
August 7, 2015: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/25917543/fdg-pet-ct-in-infection-and-inflammation-current-and-emerging-clinical-applications
#18
REVIEW
S Vaidyanathan, C N Patel, A F Scarsbrook, F U Chowdhury
Integrated positron emission tomography/computed tomography (PET/CT) with the glucose analogue, 2-[(18)F]-fluoro-2-deoxy-d-glucose (FDG), is an evolving hybrid imaging technique in the evaluation of an important and diverse group of pathological conditions, which are characterised by infection and aseptic inflammation. With a rapidly expanding body of evidence, it is being increasingly recognised that, in addition to its established role in oncological imaging, FDG PET/CT also has clinical utility in suspected infection and inflammation...
July 2015: Clinical Radiology
https://www.readbyqxmd.com/read/25403779/chromosome-18q-deletion-syndrome-with-autoimmune-diabetes-mellitus-putative-genomic-loci-for-autoimmunity-and-immunodeficiency
#19
Anna Hogendorf, Beata S Lipska-Zietkiewicz, Agnieszka Szadkowska, Maciej Borowiec, Magdalena Koczkowska, Piotr Trzonkowski, Izabela Drozdz, Krystyna Wyka, Janusz Limon, Wojciech Mlynarski
A girl with 18q deletion syndrome was diagnosed with autoimmune diabetes mellitus and Hashimoto's thyroiditis at the age of 3 yr. In addition, the girl suffered from recurrent infections due to immunoglobulin A and IgG4 deficiency. She was also found to have CD3+CD4+FoxP3+, CD3+CD4+FoxP3+CD25+, and CD3+CD4+CD25+CD127 regulatory T cells deficiency. The exceptional coincidence of the two autoimmune disorders occurring at an early age, and associated with immune deficiency, implies that genes located on deleted 19...
March 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/25339459/serum-immunoglobulins-in-28-adults-with-autoimmune-sensorineural-hearing-loss-increased-prevalence-of-subnormal-immunoglobulin-g1-and-immunoglobulin-g3
#20
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
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