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IgG 4 Related disease

Manish Soneja, S Achintya, Neeraj Nischal, Piyush Ranjan, Surabhi Vyas, Sudheer Arava, S K Sharma
A large proportion of patients with retroperitoneal fibrosis (RPF) have been labelled as having idiopathic RPF historically. Recent reports of a subset of these patients having a fibro-inflammatory condition with characteristic histology and good response to therapy has helped in renewed understanding of an old disease. A 40 year old man presented with vague abdominal discomfort of 5 months duration. Ultrasound examination revealed soft tissue thickening with calcification over lower abdominal aorta. CT scan characterized the lesion as a retroperitoneal mass encasing the aorta with an associated lesion in upper pole of left kidney...
June 2016: Journal of the Association of Physicians of India
Melanie C Bois, Hiroshi Sekiguchi, Jay H Ryu, Eunhee S Yi
Pulmonary nodular lymphoid hyperplasia (PNLH) may show heavy lymphoplasmacytic infiltrates with increased Immunoglobulin G4 (IgG4)-positive plasma cells as in IgG4-related disease (IgG4-RD). However, it is unclear whether PNLH could be a manifestation of IgG4-RD. A recent study reported the presence of Epstein-Barr virus (EBV) in IgG4-related lymphadenopathy. We postulated that a subset of PNLH might represent IgG4-related lung disease (IgG4-RLD) with EBV-positive lymphocytes as has been reported in IgG4-related lymphadenopathy...
October 5, 2016: Human Pathology
Sunuraj Sivarajan, Siddharudha Shivalli, Debomallya Bhuyan, Michael Mawlong, Rittwick Barman
BACKGROUND: India is an integral component of "tsutsugamushi triangle" which depicts a part of the globe endemic to scrub typhus. Owing to frequent outbreaks witnessed in different parts of the country in the recent past, scrub typhus is described as a re-emerging infectious disease in India. The present study aimed to study the clinical and paraclinical profile, complications and predictors of outcome among 90 cases of scrub typhus diagnosed in a hospital of north-eastern India from Sept 2011 to Aug 2012...
October 5, 2016: Infectious Diseases of Poverty
Valentina Damato, Amelia Evoli, Raffaele Iorio
Importance: Neuromyelitis optica spectrum disorders (NMOSDs) are autoimmune astrocytopathies characterized by predominant involvement of the optic nerves and spinal cord. In most patients, an IgG autoantibody binding to astrocytic aquaporin 4, the principal water channel of the central nervous system, is detected. Rituximab, a chimeric monoclonal antibody specific for the CD20 B-lymphocyte surface antigen, has been increasingly adopted as a first-line off-label treatment for patients with NMOSDs...
September 26, 2016: JAMA Neurology
Susan O'Brien, Jeffrey A Jones, Steven E Coutre, Anthony R Mato, Peter Hillmen, Constantine Tam, Anders Österborg, Tanya Siddiqi, Michael J Thirman, Richard R Furman, Osman Ilhan, Michael J Keating, Timothy G Call, Jennifer R Brown, Michelle Stevens-Brogan, Yunfeng Li, Fong Clow, Danelle F James, Alvina D Chu, Michael Hallek, Stephan Stilgenbauer
BACKGROUND: The TP53 gene, encoding tumour suppressor protein p53, is located on the short arm of chromosome 17 (17p). Patients with 17p deletion (del17p) chronic lymphocytic leukaemia have poor responses and survival after chemoimmunotherapy. We assessed the activity and safety of ibrutinib, an oral covalent inhibitor of Bruton's tyrosine kinase, in relapsed or refractory patients with del17p chronic lymphocytic leukaemia or small lymphocytic lymphoma. METHODS: We did a multicentre, international, open-label, single-arm study at 40 sites in the USA, Canada, Europe, Australia, and New Zealand...
October 2016: Lancet Oncology
Michelle Shui Yee Lau, Peter Mooney, William White, Victoria Appleby, Sulleman Moreea, Ismail Haythem, Joshua Elias, Kiran Bundhoo, Gareth Corbett, Liam Wong, Her Hsin Tsai, Simon Cross, John Hebden, Sami Hoque, David Sanders
BACKGROUND: International guidelines recommend coeliac serology in iron deficiency anaemia, and duodenal biopsy for those tested positive to detect coeliac disease. However, pre-endoscopy serology is often unavailable, thus committing endoscopists to take routine duodenal biopsies. Some endoscopists consider duodenal biopsy mandatory in anaemia to exclude other pathologies. We hypothesise that using a point of care test at endoscopy could fill this gap, by providing rapid results to target anaemic patients who require biopsies, and save costs by biopsy avoidance...
September 15, 2016: BMC Gastroenterology
Nicholas Horton, Xianrui Wu, Jessica Philpott, Ari Garber, Jean-Paul Achkar, Aaron Brzezinski, Bret A Lashner, Bo Shen
BACKGROUND: Inflammatory bowel diseases (IBDs) are considered immune-mediated disorders with dysregulated innate and adaptive immunities. Secondary immunogloblin deficiency can occur in IBD and its impact on the disease course of IBD is not clear. AIMS: We sought to determine associations between low IgG/G1 levels and poor clinical outcomes in IBD patients. METHODS: This historic cohort study was performed on IBD patients with obtained IgG/IgG1 levels...
November 2016: Digestive Diseases and Sciences
M Borte, G Kriván, B Derfalvi, L Maródi, T Harrer, S Jolles, C Bourgeois, W Engl, H Leibl, B McCoy, D Gelmont, L Yel
A highly concentrated (20%) immunoglobulin (Ig)G preparation for subcutaneous administration (IGSC 20%), would offer a new option for antibody replacement therapy in patients with primary immunodeficiency diseases (PIDD). The efficacy, safety, tolerability and pharmacokinetics of IGSC 20% were evaluated in a prospective trial in Europe in 49 patients with PIDD aged 2-67 years. Over a median of 358 days, patients received 2349 IGSC 20% infusions at monthly doses equivalent to those administered for previous intravenous or subcutaneous IgG treatment...
September 10, 2016: Clinical and Experimental Immunology
Wayne R Thomas
PURPOSE OF REVIEW: Recent findings on house dust allergens and their contribution to knowledge that will significantly impact on current and future allergy treatments are appraised. RECENT FINDINGS: Quantitation of IgE binding to a spectrum of allergen components in several independent studies in varying locations has largely affirmed the main components as the groups 1 and 2 and possibly 23 allergens with mid-tier contributions from the groups 4, 5, 7, and 21. Prevalent binding to Der p 23 has been recapitulated sometimes with low titers...
September 2016: Current Allergy and Asthma Reports
Toan Nguyen, Fei Wen Cheong, Jonathan Wee Kent Liew, Yee Ling Lau
BACKGROUND: Despite the global effort against neglected tropical diseases (NTDs), developing countries with middle to low income are still burdened by them. Vietnam has been undergoing substantial economic growth and urbanization, but underprivileged people living in rural and suburban areas are still having little access to public health infrastructure and proper sanitation. Hitherto, limited information is available for seroprevalence and risk factors of several parasitic diseases in Vietnam...
2016: Parasites & Vectors
Yanbing Wang, Hong Yan, Qixing Ding, Cunhua Mao, Yelong Shen, Guangbin Wang
Neuromyelitis optica (NMO) is an acute or subacute lesion of demyelinating disease involving the optic nerve and spinal cord, and imaging techniques and their effects have been the focus of investigations. The aim of the present study was to examine the value of three-dimensional double inversion recovery (3D-DIR) in the early differential diagnostic and prognostic evaluation of NMO. Forty-eight patients with suspicious NMO were included into the study and underwent a combination of serum NMO-IgG quantitative detection and 3D-DIR examination...
September 2016: Experimental and Therapeutic Medicine
Daniel Suez, Mark Stein, Sudhir Gupta, Iftikhar Hussain, Isaac Melamed, Kenneth Paris, Amy Darter, Christelle Bourgeois, Sandor Fritsch, Heinz Leibl, Barbara McCoy, David Gelmont, Leman Yel
Patients with primary immunodeficiency disease (PIDD) typically require life-long intravenous (IV) or subcutaneous (SC) immunoglobulin (Ig) replacement therapy to prevent recurrent infections. The efficacy, safety, and pharmacokinetics of a highly concentrated (20 %) Ig preparation for SC administration (IGSC 20 %) were evaluated in a prospective trial in patients with PIDD. A total of 74 patients (aged 3-83 years) received 4327 IGSC 20 % infusions over a median of 380.5 days. The rate of validated serious bacterial infections was 0...
October 2016: Journal of Clinical Immunology
Guozhi Xia, Xin Sun, Xiaopu Zheng, Junkui Wang
Peripartum cardiomyopathy (PPCM) is a disease of unknown pathogenesis. Programmed death 1 (PD1) has been postulated to modulate immune response through potential mechanisms that remain elusive. This study aimed to elaborate the expression and function of PD1 on peripheral blood lymphocytes (PBLs) in the development of PPCM. Specimens of PBLs were performed to determine the expression of PD1 mRNA using fluorescence quantitative RT-PCR, and Th cytokines by ELISA. Immune homeostasis was evaluated with T lymphocyte phenotypes and immunoglobulin (Ig) isotypes as well as complement factors (C)...
November 15, 2016: International Journal of Cardiology
T N K W Gunasekara, S Di Palma, I N Bagwan
BACKGROUND: IgG4 related disease rarely affects the salivary glands and clinically is often confused with salivary gland malignancy. METHOD: This is a retrospective study comprising 137 cases of chronic sialadenitis diagnosed in a histopathology department over 4 years. The morphology was assessed by reviewing the histology slides and the incidence of IgG4 related sclerosing sialadenitis was calculated. IgG and IgG4 immunohistochemistry was performed and mean IgG4 count/hpf and IgG4/IgG ratio were determined...
August 2016: Malaysian Journal of Pathology
Endrias Zewdu Gebremedhin, Nura Dima, Ashenafi Feyisa Beyi, Fufa Dawo, Negassa Feyissa, Edilu Jorga, Vincenzo Di Marco, Maria Vitale
Toxoplasmosis is one of the most prevalent parasitic infections of medical and veterinary importance. A cross-sectional study was conducted from November 2013 to January 2014 to estimate the seroprevalence of Toxoplasma gondii infection in camels from four districts of Borana zone, Southern Oromia, Ethiopia. In addition, a questionnaire survey was administered to 124 pastoralists to identify possible risk factors and to assess the awareness level of pastoral communities about toxoplasmosis. A total of 396 serum samples were examined for anti-Toxoplasma IgG antibodies using the direct agglutination test (DAT)...
August 24, 2016: Tropical Animal Health and Production
Kung-Chao Chang, Jen-Chieh Lee, Yu-Chu Wang, Liang-Yi Hung, Yenlin Huang, Wan-Ting Huang, Ren-Ching Wang, Tse-Ching Chen, Yi-Shan Tsai, L Jeffrey Medeiros
Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a morphologically distinctive lesion. Although the clinical course of SANT is benign, its reactive or neoplastic nature remains to be clarified. Furthermore, some investigators have suggested that SANT is related to IgG4 sclerosing lesion or inflammatory pseudotumor with stromal cells positive for Epstein-Barr virus (EBV). In this study, we assessed 22 cases of SANT derived from adult women. Clinical data and follow-up information were obtained by chart review...
October 2016: American Journal of Surgical Pathology
Nicholas Kavana, Parthasarathy Sonaimuthu, Christopher Kasanga, Ayub Kassuku, Hesham M Al-Mekhlafi, Mun Yik Fong, Mohammad Behram Khan, Rohela Mahmud, Yee Ling Lau
In this study, the seroprevalence of sparganosis and its relationship with sociodemographic factors in northern Tanzania have been assessed. A total of 216 serum samples from two rural districts, Monduli and Babati, were tested for sparganosis using an enzyme-linked immunosorbent assay. The seroprevalence of anti-sparganum IgG antibodies was 62.5% (95% confidence interval [CI] = 56.1-68.9) in all age groups. There were significant associations between district (relative risk [RR] = 1.95, 95% CI = 1.42-2.69), education (RR = 1...
October 5, 2016: American Journal of Tropical Medicine and Hygiene
Ji-Sun Kim, Soyoung Cheon, Seung Woo Kim, Boram Kim, Heejaung Kim, Ki Duk Park, Sung-Min Kim
Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system and is mediated by complement-dependent cytotoxicity (CDC) of NMO-specific immunoglobulin G (IgG) antibodies (NMO-IgG). Glycyrrhizic acid (GA) has numerous pharmacological effects including inhibition of the complement pathway. We aimed to study the influence of GA on NMO-IgG-induced CDC. NMO-IgG samples from 7 patients with NMO, together with human complement, induced CDC in an aquaporin 4 M23-overexpressing glial cell line, an in vitro NMO model...
September 16, 2016: Biochemical and Biophysical Research Communications
Akihiro Manabe, Takuro Igawa, Mai Takeuchi, Yuka Gion, Tadashi Yoshino, Yasuharu Sato
Plasma cell-type Castleman disease (PCD) is often encountered when differentiating IgG4-related disease (IgG4-RD). Given that serum IgA is often elevated in Castleman disease, we investigated whether IgA expression levels in histological specimens can be used to differentiate between the two diseases. Lymph node lesions obtained from 12 IgG4-RD and 11 PCD patients were analysed by immunohistochemistry with anti-IgG, -IgG4, and -IgA antibodies. In addition to all 12 cases of IgG4-RD, 8/11 cases (72.7 %) of PCD also met the diagnostic criteria of IgG4-RD (serum IgG4 ≥135 mg/dl and IgG4/IgG-positive cells ≥40 %)...
July 20, 2016: Medical Molecular Morphology
Yu Chen, Ji-Zhi Zhao, Rui-E Feng, Ju-Hong Shi, Xue-Mei Li, Yun-Yun Fei, Yang Shi, Wen Zhang, Feng-Chun Zhang
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic disease that can involve multiple organs and various clinical phenotypes. The purpose of this study was to analyze different types of organ involvement in IgG4-RD patients in China. METHODS: We conducted a prospective cohort study on IgG4-RD patients to analyze the clinical manifestations and rare features of IgG4-RD. Patients were grouped into different types according to organ involvement regarding organ number and organ site...
July 5, 2016: Chinese Medical Journal
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