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IgG4 disease

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https://www.readbyqxmd.com/read/29790577/esophageal-igg4-levels-correlate-with-histopathologic-and-transcriptomic-features-in-eosinophilic-esophagitis
#1
Chen E Rosenberg, Melissa K Mingler, Julie M Caldwell, Margaret H Collins, Patricia C Fulkerson, David W Morris, Vincent A Mukkada, Philip E Putnam, Tetsuo Shoda, Ting Wen, Marc E Rothenberg
BACKGROUND: Recent data associate eosinophilic esophagitis (EoE) with IgG4 rather than IgE, but its significance and function have not been determined. Our aims were to measure esophageal IgG4 levels and to determine functional correlations as assessed by histologic and transcriptome analyses. METHODS: This case-control study included pediatric subjects with EoE (≥ 15 eosinophils/HPF) and non-EoE controls. Protein lysates were analyzed for IgA, IgM, and IgG1-IgG4 using the Luminex 100 system; IgE was quantified by ELISA...
May 23, 2018: Allergy
https://www.readbyqxmd.com/read/29790305/lyme-disease-associated-igg4-autoantibodies-correlate-with-synovial-pathology-in-antibiotic-refractory-lyme-arthritis
#2
Katherine B Sulka, Klemen Strle, Jameson T Crowley, Robert B Lochhead, Robert Anthony, Allen C Steere
OBJECTIVE: To determine whether IgG subclasses of B. burgdorferi antibodies differ from those of 3 Lyme disease (LD)-associated autoantibodies. METHODS: The IgG antibody subclasses were determined by ELISA in serum samples from 215 patients representative of each of the 3 stages of Lyme disease. Antibody and cytokine profiles were measured in matched serum and synovial fluid (SF) samples from Lyme arthritis patients. Synovial tissue from patients with antibiotic-refractory arthritis was examined for histologic features, IgG subclasses of plasma cells, and mRNA subclass expression...
May 23, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29781221/aberrant-expansion-and-function-of-t-follicular-helper-cell-subsets-in-igg4-related-disease
#3
Yu Chen, Wei Lin, Hongxian Yang, Mu Wang, Panpan Zhang, Ruie Feng, Hua Chen, Linyi Peng, Xuan Zhang, Yan Zhao, Xiaofeng Zeng, Fengchun Zhang, Wen Zhang, Peter E Lipsky
OBJECTIVE: To determine the number and function of Tfh cell subsets in IgG4-RD. METHODS: Mononuclear cells from peripheral blood and involved tissues of IgG4-RD patients were collected. Tfh cell subsets, mRNA levels of Bcl-6, Blimp-1 and IL-21 were tested. Immunohistochemistry and immunofluorescence techniques were used to assess the location of IL-21, Bcl-6, and (CD4+CXCR5+) Tfh cells in the involved tissues. Furthermore, by in vitro cell co-culture, the abilities of cTfh cell subsets to induce B cells proliferation, apoptosis, differentiation, and production of IgG4 were explored...
May 21, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29780105/a-case-of-longstanding-igg4-related-ophthalmic-disease-dramatically-improved-after-steroid-therapy
#4
Yoko Hirata, Jiro Fukae, Akihiro Nishida, Shinsuke Fujioka, Mokoto Hamasaki, Masanari Nonokuma, Yoshio Tsuboi
A 61-year-old man was admitted to our hospital because of decreased visual acuity. On admission, he had marked blepharoedema, conjunctival injection, exophthalmos, diplopia, and blurred vision. He also had bronchial asthma and urinary retention requiring urethral catheterization. His serum immunoglobulin (Ig) G4 level was elevated to 1,830 U/mL. Fluorodeoxyglucose-positron emission tomography revealed an abnormal uptake in multiple organs. A histopathological examination of the salivary gland revealed IgG4-positive plasma cell infiltration, leading to a diagnosis of IgG4-related ophthalmic disease...
May 18, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29774578/the-immunobiology-of-rankl-and-myeloid-derived-suppressor-cell-activation-in-igg4-related-sclerosing-cholangitis
#5
Min Lian, Qixia Wang, Xiang Jiang, Jun Zhang, Yiran Wei, Yanmei Li, Bo Li, Weihua Chen, Haiyan Zhang, Qi Miao, Yanshen Peng, Xiao Xiao, Li Sheng, Weici Zhang, Jingyuan Fang, Ruqi Tang, M Eric Gershwin, Xiong Ma
The primary function of myeloid derived suppressor cells (MDSCs) is reflected in their immune modulatory role in several immune-mediated diseases. In IgG4-related disease (IgG4-RD), it has been hypothesized that there are selective regulatory defects that lead to a Th2 bias immune response. Herein we have taken advantage of a large cohort of patients with IgG4-related sclerosing cholangitis (IgG4-SC), the most common extra-pancreatic involvement of IgG4-RD, as well as controls consisting of primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH) and healthy volunteers, to study MDSC...
May 18, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29774301/-mansonella-perstans-onchocerca-volvulus-and-strongyloides-stercoralis-infections-in-rural-populations-in-central-and-southern-togo
#6
Francois Korbmacher, Kossi Komlan, Richard G Gantin, Wiyao P Poutouli, Koffi Padjoudoum, Potchoziou Karabou, Peter T Soboslay, Carsten Köhler
Background: Mansonella perstans , Onchocerca volvulus and Strongyloides stercoralis are widespread helminth parasites in the tropics. Their distribution remains difficult to determine as it may change during national disease control programs and with regional mass drug administration (MDA). Epidemiological surveys are of importance to evaluate the geographical distribution of these helminth parasites and the diseases they may cause, however, up to date epidemiological evaluations on M...
May 2018: Parasite Epidemiology and Control
https://www.readbyqxmd.com/read/29768335/igg4-related-disease-of-pulmonary-artery-causing-pulmonary-hypertension
#7
Hui Deng, Sheng Zhao, Yunlong Yue, Yong Liu, Yali Xu, Jin Qian, Xiaorong Ma, Peiliang Gao, Xiaoyan Yao, Xin Jiang, Xiqi Xu, Zhicheng Jing, Yong Wang, Lei Pan, Xinying Xue
IgG4-related disease (IgG4-RD) is recognized as an immune-mediated condition with pathology features of lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis, accompanied with or without elevated serum IgG4 concentrations. However, few of pulmonary artery IgG4-RD causing pulmonary hypertension (PH) was reported.The medical records of 3 patients with pulmonary artery IgG4-RD inducing PH were analyzed retrospectively.Imaging findings demonstrated that the lesions of 3 patients located in pulmonary artery, which were initially diagnosed as pulmonary thrombus or malignant tumor...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29767269/-igg4-associated-cholangitis-clinical-presentation-of-an-overlooked-disease-entity
#8
REVIEW
T Herta, J Verheij, U Beuers
IgG4-associated cholangitis (IAC) is the hepatobiliary manifestation of immunoglobulin G4-related disease, which is an immune-mediated fibroinflammatory systemic disorder characterised by often elevated IgG4 serum levels and typical histopathological findings in affected tissues. IAC is frequently (>90%) accompanied by autoimmune pancreatitis type 1 (AIP), which is the pancreatic manifestation of immunoglobulin G4-related disease. In 80-85% of the cases patients with IAC are male, above 50 years of age and present with jaundice and weight loss...
May 16, 2018: Der Internist
https://www.readbyqxmd.com/read/29766732/-remission-of-the-disease-associated-related-with-immunoglobulin-igg4-accompanied-by-multiple-lymphadenopathy-after-treatment-with-rituximab-and-dexamethasone-a-case-report
#9
Zdeněk Adam, Zita Chovancová, Markéta Nová, Pavel Fabian, Zdeněk Řehák, Renata Koukalová, Marek Slávik, Luděk Pour, Marta Krejčí, Aleš Čermák, Zdeněk Král, Jiří Mayer
A disease associated with immunoglobulin IgG4 is a rare unit with very variable symptoms. We describe the course and treatment of the disease in a patient who presented with multiple lymphadenopathy and infiltrates in the area of the retroperitoneum and pelvis and signs of chronic sclerosing pancreatitis. The disease was clinically manifested by a significant loss of weight, but also by a loss of perception of taste and smell. The diagnosis was made based on a high amount of IgG4 expressing plasma cells in the sampled tissue and an increased concentration of immunoglobulins of type IgG and mainly subclass IG4...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29765543/igg4-related-kidney-disease-the-effects-of-a-rituximab-based-immunosuppressive-therapy
#10
Giacomo Quattrocchio, Antonella Barreca, Andrea Demarchi, Laura Solfietti, Giulietta Beltrame, Roberta Fenoglio, Michela Ferro, Paola Mesiano, Stefano Murgia, Giulio Del Vecchio, Carlo Massara, Cristiana Rollino, Dario Roccatello
IgG4-related disease (IgG4-RD) is a recently recognized disorder, characterized by elevated serum IgG4 concentrations, dense tissue infiltration of IgG4-positive plasma cells and storiform fibrosis. Treatment is usually based on steroids, however, relapses and long-term adverse effects are frequent. We prospectively studied 5 consecutive patients with histologically-proven IgG4-RD and renal involvement, treated with an extended Rituximab protocol combined with steroids. Two doses of intravenous cyclophosphamide were added in 4 patients...
April 20, 2018: Oncotarget
https://www.readbyqxmd.com/read/29762245/igg4-related-disease-simulating-carcinoma-colon-with-diffuse-peritoneal-carcinomatosis-on-18f-fdg-pet-ct
#11
Shelvin Kumar Vadi, Ashwin Singh Parihar, Rajender Kumar, Harmandeep Singh, Bhagwant Rai Mittal, Amanjit Bal, Saroj Kumar Sinha
IgG4-related disease (IgG4-RD) continues to be a diagnostic challenge and a great mimicker of malignancies. We report here a case of young man who presented with subacute intestinal obstruction with initial imaging and clinical features suggestive of carcinoma colon. 18F-FDG PET/CT showed diffuse peritoneal carcinomatosis pattern typically seen with abdominal malignancies. However, the histopathology and the raised IgG4 levels diagnosed it to be IgG4-RD. Although 18F-FDG PET/CT has typical patterns corresponding to the multisystemic involvement of IgG4-RD, the index case did not show any such findings...
May 14, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29759137/low-amounts-of-bisecting-glycans-characterize-cerebrospinal-fluid-borne-igg
#12
Jasmin Knopf, Iryna Magorivska, Juan M Maler, Philipp Spitzer, Rostyslav Bilyy, Mona H C Biermann, Kateryna Hychka, Albert Bondt, Manfred Wuhrer, Rene E M Toes, Georg Schett, Martin Herrmann, Luis E Muñoz
Immunoglobulin G (IgG) harbors a conserved N-glycosylation site which is important for its effector functions. Changes in glycosylation of IgG occur in many autoimmune diseases but also in physiological conditions. Therefore, the glycosylation pattern of serum IgG is well characterized. However, limited data is available on the glycosylation pattern of IgG in cerebrospinal fluid (CSF) compared to serum. Here, we report significantly reduced levels of bisected glycans in CSF IgG. Galactosylation and sialylation of IgG4 also differed significantly...
July 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29755523/hypophyseal-involvement-in-immunoglobulin-g4-related-disease-a-retrospective-study-from-a-single-tertiary-center
#13
Yang Liu, Linjie Wang, Wen Zhang, Hui Pan, Hongbo Yang, Kan Deng, Lin Lu, Yong Yao, Shi Chen, Xiaofeng Chai, Feng Feng, Hui You, Zimeng Jin, Huijuan Zhu
This study aims to outline the clinical features and outcomes of IgG4-related hypophysitis (IgG4-RH) patients in a tertiary medical center. We reviewed clinical manifestations and imaging and pituitary function tests at baseline, as well as during follow-up. Ten patients were included. The mean age at diagnosis of IgG4-RH was 48.4 (16.0-64.0) years. An average of 3 (0-9) extrapituitary organs were involved. Five patients had panhypopituitarism, three had only posterior hypopituitarism, one had only anterior hypopituitarism, and one had a normal pituitary function...
2018: International Journal of Endocrinology
https://www.readbyqxmd.com/read/29755273/orbital-progressive-transformation-of-germinal-centers-as-part-of-the-spectrum-of-igg4-related-ophthalmic-disease-clinicopathologic-features-of-three-cases
#14
M Adelita Vizcaino, Shannon S Joseph, Charles G Eberhart
Progressive transformation of germinal centers (PTGC) is a form of follicular hyperplasia recently associated with immunoglobulin G4-related disease (IgG4-RD), but the ophthalmic manifestations of this combination are poorly described. In this retrospective case series, we present three cases of IgG4-related orbital disease (IgG4-ROD) showing varying degrees of PTGC involving the orbit and lacrimal gland. Three adult women presented with ill-defined lacrimal gland enlargement. Histologic sections showed variable fibrosis and large, irregular lymphoid follicles with prominent mantle zones penetrating the germinal centers, highlighted by Bcl-2 and/or IgD immunostains...
January 2018: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/29754327/the-many-faces-of-igg4-related-disease-report-of-a-case-with-inaugural-recurrent-aortic-aneurism-ruptures-and-literature-review
#15
REVIEW
Mariana Luís, Luísa Brites, Bruno Fernandes, Diogo Jesus, Tânia Santiago, Sara Serra, João Rovisco, Lina Carvalho, José António P da Silva, Armando Malcata
Vascular involvement in IgG4-related disease (IgG4-RD), is a well-recognized feature and large vessel commitment, especially the aorta, can be the only manifestation of the disease. Being a newly recognized disease, its diagnosis and workup still represents a challenge in clinical practice. A 47-year-old-man with two aortic aneurysms ruptures, one at abdominal and the other at thoracic level, was referred to our rheumatology department. The initial analysis of the surgical specimen obtained 3 years earlier revealed a nonspecific aortitis...
May 12, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29749030/a-new-therapeutic-approach-for-type-1-diabetes-rationale-for-gnbac1-an-anti-herv-w-env-monoclonal-antibody
#16
REVIEW
Francois Curtin, Corinne Bernard, Sandrine Levet, Hervé Perron, Hervé Porchet, Julie Médina, Sam Malpass, David Lloyd, Richard Simpson
We describe a newly identified therapeutic target for type 1 diabetes: an envelope protein of endogenous retroviral origin called Human Endogenous Retrovirus W Envelope (HERV-W-Env). HERV-W-Env was found to be detected in the blood of around 60% of type 1 diabetes (T1D) patients and is expressed in acinar pancreatic cells of 75% of T1D patients at post-mortem examination. Preclinical experiments showed that this protein displays direct cytotoxicity on human β-islet cells. In vivo HERV-W-Env impairs the insulin and glucose metabolism in transgenic mice expressing HERV-W-Env...
May 10, 2018: Diabetes, Obesity & Metabolism
https://www.readbyqxmd.com/read/29742724/a-case-report-of-immunoglobulin-g4-related-sclerosing-cholangitis-with-multiple-relapse
#17
Xiaoqin Dong, Na Huo, Zhao Wu, Guiqiang Wang, He Wang, Hong Zhao
RATIONALE: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is classified as a biliary tract manifestation of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoid is the first-line therapy for most patients, but the optimal starting dose, adequate maintaining dose and withdrawal time remain disputable. PATIENT CONCERNS: An elderly male patient presented to our hospital with neoplasms of the bile duct and pancreas at first visit in December 2011...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29741204/striatal-hypermetabolism-in-a-case-of-igg4-related-disease
#18
Abdelhamid Biyi, Omar Ait Sahel, Abderrahim Mejjad, Yasser Benameur, Salah Nabih Oueriagli, Abderrahim Doudouh
A 77 years-old man with lung and bone involvement of proven IgG4-related disease complained of tree month's memory deficits. Brain MRI was normal. 18F-FDG whole body PET/CT showed in addition to the bone lesions, a marked symmetrical striatal hypermetabolism in contrast with cortical hypometabolism. Despite steroid treatment, the patient's clinical status declined rapidly and he died two months later. Striatal hypermetabolism has been reported in autoimmune limbic encephalitis, but to the best of our knowledge, this is the first description in a case of IgG4-related disease...
2018: Nuclear Medicine Review. Central & Eastern Europe
https://www.readbyqxmd.com/read/29738364/a-newly-recognized-histologic-pattern-of-igg4-related-lymphadenopathy-expanding-the-morphologic-spectrum
#19
Ying-Ren Chen, Yi-Ju Chen, Ming-Chung Wang, L Jeffrey Medeiros, Kung-Chao Chang
Immunoglobulin (Ig)G4-related sclerosing disease is a fibroinflammatory disorder characterized by tumor-forming lesions at multiple anatomic sites and by increased serum levels of IgG4. IgG4-related lymphadenopathy, defined as lymphadenopathy developing in patients with IgG4-related sclerosing disease, is known to manifest in 5 histologic patterns: (1) multicentric Castleman disease-like; (2) reactive follicular hyperplasia; (3) interfollicular plasmacytosis with immunoblasts; (4) progressive transformation of germinal centers-like; and (5) inflammatory pseudotumor-like...
May 4, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29737040/marked-accumulation-of-fdg-and-inflammatory-cells-expressing-glucose-transporter-3-in-igg4-related-autoimmune-hepatitis
#20
Toshihiro Araki, Teruko Arinaga-Hino, Hironori Koga, Jun Akiba, Tatsuya Ide, Yoshinobu Okabe, Reiichiro Kuwahara, Keisuke Amano, Makiko Yasumoto, Toshihiro Kawaguchi, Tomoya Sano, Reiichiro Kondou, Seiji Kurata, Keiichi Mitsuyama, Takuji Torimura
Immunoglobulin G (IgG) 4 related-autoimmune hepatitis (AIH) is a recently proposed subtype that responds well to steroid treatment; however, its pathogenesis remains unclear. We report here a 65-year-old Japanese female with skin itching and lip swelling. She had liver injury with jaundice, which persisted despite stopping of anti-allergic agents. Blood chemistry revealed highly elevated serum IgG and IgG4 (535 mg/dL) levels, and positive anti-nuclear antibody. The diagnosis of AIH was based on liver biopsy...
May 7, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
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