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"IgG4-related disease"

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https://www.readbyqxmd.com/read/29781221/aberrant-expansion-and-function-of-t-follicular-helper-cell-subsets-in-igg4-related-disease
#1
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
#2
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
#3
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/29768335/igg4-related-disease-of-pulmonary-artery-causing-pulmonary-hypertension
#4
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/29765543/igg4-related-kidney-disease-the-effects-of-a-rituximab-based-immunosuppressive-therapy
#5
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
#6
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/29754327/the-many-faces-of-igg4-related-disease-report-of-a-case-with-inaugural-recurrent-aortic-aneurism-ruptures-and-literature-review
#7
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/29741204/striatal-hypermetabolism-in-a-case-of-igg4-related-disease
#8
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/29736618/leptomeningeal-form-of-immunoglobulin-g4-related-hypertrophic-meningitis-with-perivascular-spread-a-case-report-and-review-of-the-literature
#9
Jasmina Boban, Selin Ardalı, Majda M Thurnher
PURPOSE: Immunoglobulin G4 (IgG4)-related disease represents a spectrum of fibro-inflammatory disorders that affects various organ systems, including the central nervous system. METHODS: Here we present the case of lgG4-related hypertrophic meningitis with exclusively leptomeningeal involvement and spread via perivascular spaces. RESULTS: A 58-year-old male patient presented with complex partial seizures. Initial computed tomography examination showed left frontal sulcal hyperdensity...
May 7, 2018: Neuroradiology
https://www.readbyqxmd.com/read/29736428/early-treatment-for-igg4-related-disease-may-prevent-cognitive-impairment-caused-by-cerebral-vasculitis-a-case-report-and-review-of-the-literature
#10
Toshihiko Usami, Shoji Kawashima, Yoshino Ueki, Takanari Toyoda, Kenji Okita, Noriyuki Matsukawa
IgG4-related disease (IgG4-RD) is a recently recognized disease entity. A 74-year-old male presented with transient headache. He was diagnosed IgG4-RD by pancreatic biopsy at the age of 72. Magnetic Resonance Imaging (MRI) showed disseminated cerebral microbleeds and microinfarctions in time and space. It suggested cerebral vasculitis, however any causative factor were not confirmed. IgG4-RD rarely causes cerebral vasculitis. This might be a first case of an asymptomatic cerebral vasculitis due to IgG4-RD. Patient was started on oral prednisolone, and no neurological or neuropsychological symptom was clinically observed...
March 2018: ENeurologicalSci
https://www.readbyqxmd.com/read/29736303/hypothyroidism-in-patients-with-autoimmune-pancreatitis
#11
Ryoko Shimizuguchi, Terumi Kamisawa, Yuka Endo, Masataka Kikuyama, Sawako Kuruma, Kazuro Chiba, Taku Tabata, Satomi Koizumi
AIM: To examine thyroid function and clinical features of hypothyroidism in autoimmune pancreatitis (AIP) patients. METHODS: We examined thyroid function in 77 patients with type 1 AIP (50 males, 27 females; median age 68 years, range 33-85) diagnosed according to the Japanese diagnostic criteria for AIP 2011. We compared clinical and serological findings between patients with and without various categories of hypothyroidism. The change in hypothyroidism after steroid therapy was also examined...
May 6, 2018: World Journal of Gastrointestinal Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/29732477/effectiveness-of-rituximab-containing-treatment-regimens-in-idiopathic-multicentric-castleman-disease
#12
Yujun Dong, Lu Zhang, Lin Nong, Lihong Wang, Zeyin Liang, Daobin Zhou, David C Fajgenbaum, Hanyun Ren, Jian Li
Human herpes virus type 8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disease often involving constitutional symptoms, cytopenias, and multiple organ system dysfunction. In China, the majority of MCD cases are HHV-8 negative. Given that siltuximab, the only FDA-approved treatment for iMCD is not available in China; rituximab- and cyclophosphamide-containing regimens are often used in the treatment of Chinese iMCD patients. To evaluate the efficacy of rituximab in this rare and heterogeneous disease, clinical and pathological data from 27 cases of iMCD were retrospectively analyzed from two large medical centers in China...
May 7, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29704388/unrecognised-igg4-association-in-progressively-transformed-germinal-centers-of-lymph-nodes-with-subsequent-full-blown-igg4-related-chronic-fibrosing-pancreatitis-a-case-report
#13
H J Son, H Lee, J H Kim, I K Yu, H Y Han
Progressively transformed germinal centers (PTGC) is a benign process characterised by a morphological variant of reactive follicular hyperplasia in lymph nodes. It was recently shown that some cases of PTGC are associated with IgG4-related disease (IgG4-RD) or increased IgG4 plasma cells. Five years ago, a 57-year-old woman presented with enlargement of multiple lymph nodes in the left parotid, submandibular, and neck areas, pathologically diagnosed as PTGC after excisional biopsy. Since then, she has experienced numbness in her extremities, especially the left shoulder and arm, pruritus on the left side of the face and intermittent facial palsy, for which she has been receiving regular symptomatic treatment...
April 2018: Malaysian Journal of Pathology
https://www.readbyqxmd.com/read/29701393/igg4-disease-and-slerosing-aortitis
#14
Bruno Fernandes, Rui Anacleto, Lina Carvalho
INTRODUCTION: IgG4-related disease (IgG4-RD) is an immune- -mediated fibro-inflammatory condition with unknown etiology that can affect various organs. Although its prevalence is still unknown, it appears to be more frequent in adult males. Cardiovascular manifestations are rare and can include idiopathic retroperitoneal periaortic fibrosis, inflammatory aortic aneurism, inflammatory periarteritis and inflammatory pericarditis. Vascular involvement is a well-recognized feature and large vessel commitment, especially the aorta, can be the only manifestation of the disease...
July 2017: Revista Portuguesa de Cirurgia Cardio-torácica e Vascular
https://www.readbyqxmd.com/read/29696607/abdominal-manifestations-of-igg4-related-disease-a-pictorial-review
#15
REVIEW
Christopher Siew Wai Tang, Nishanth Sivarasan, Nyree Griffin
In the last decade, autoimmune pancreatitis has become recognised as part of a wider spectrum of IgG4-related disease, typically associated with elevated serum IgG4 levels and demonstrating a response to corticosteroid therapy. Radiologically, there is imaging overlap with other benign and neoplastic conditions. This pictorial review discusses the intra-abdominal manifestations of this disease on cross-sectional imaging before and after steroid treatment and the main radiological features which help to distinguish it from other key differentials...
April 25, 2018: Insights Into Imaging
https://www.readbyqxmd.com/read/29686532/soluble-interleukin-2-receptor-a-potential-marker-for-monitoring-disease-activity-in-igg4-related-disease
#16
A F Karim, L E M Eurelings, R D Bansie, P M van Hagen, J A M van Laar, W A Dik
Background: IgG4-related disease (IgG4-RD) is a fibroinflammatory condition. T-cells play a crucial role in the pathogenesis, and therefore, serum soluble interleukin-2 receptor (sIL-2R) may be a potential biomarker. Method: We studied the levels of sIL-2R in 26 histologically proven IgG4-RD patients with available serum sIL-2R and compared them to those in newly diagnosed and untreated sarcoidosis patients ( n = 78) and controls ( n = 101) and the serum sIL-2R levels in patients after treatment of IgG4-RD ( n = 15)...
2018: Mediators of Inflammation
https://www.readbyqxmd.com/read/29672582/changes-in-n-glycans-of-igg4-and-its-relationship-with-the-existence-of-hypocomplementemia-and-individual-organ-involvement-in-patients-with-igg4-related-disease
#17
Naoki Konno, Mitsuru Sugimoto, Tadayuki Takagi, Makiko Furuya, Tomoyuki Asano, Shuzo Sato, Hiroko Kobayashi, Kiyoshi Migita, Yoshiaki Miura, Taichi Aihara, Atsushi Komatsuda, Hiromasa Ohira, Hiroshi Watanabe
BACKGROUND: Although increased serum IgG4 level and tissue infiltration of IgG4-positive cells are key events in IgG4-related disease (IgG4RD), and nearly half of IgG4RD patients show hypocomplementemia, the role of IgG4 in the pathogenesis of IgG4RD remains unclear. Many reports show that altered IgG glycosylation, especially IgG with agalactosylated N-linked glycan (G0 N-glycan), have proinflammatory roles including complement activation, implicated in the pathogenesis of various inflammatory diseases...
2018: PloS One
https://www.readbyqxmd.com/read/29672431/case-of-primary-isolated-subconjunctival-igg4-related-disease
#18
Hyo Seok Lee, Won Choi, Ga Eon Kim, Kyung Chul Yoon
PURPOSE: To report a case of isolated subconjunctival ocular adnexal IgG4-related disease that met the diagnostic criteria according to the Japanese Ministry of Health, Labour and Welfare's 2011 guidelines. METHODS: We report a case of a 56-year-old woman with a left subconjunctival mass for 3 months. Excisional biopsy was performed. Postoperatively, the patient underwent systemic and radiologic evaluations for IgG4-related disease. RESULTS: The clinicopathologic study revealed storiform fibrosis and lymphoplasmacytic infiltration, with increased IgG4-positive plasma cells and an IgG4/IgG-positive plasma cell ratio of 40%...
April 18, 2018: Cornea
https://www.readbyqxmd.com/read/29666725/elevated-serum-igg4-levels-in-a-young-patient-with-polyserositis-and-necator-americanus-infection
#19
Giuseppe D Sanna, Roberto Manetti, Valentina de Filippo, Sergio Babudieri
IgG4-related disease is a fibroinflammatory systemic condition characterized by tumefactive lesions, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and elevated serum IgG4 concentrations. It has been described in virtually every organ system. Autoimmunity and infectious agents are potential immunologic triggers in IgG4-related disease. Herein, we describe a peculiar case of effusive-constrictive pericarditis in an 18-year-old boy with polyserositis and concomitant Necator americanus infection...
2018: Case Reports in Immunology
https://www.readbyqxmd.com/read/29629225/igg4-related-disease-in-the-stomach-which-was-confused-with-gastrointestinal-stromal-tumor-gist-two-case-reports-and-review-of-the-literature
#20
Ho Seok Seo, Yoon Ju Jung, Cho Hyun Park, Kyo Young Song, Eun Sun Jung
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disorder characterized by specific pathological findings and elevated serum IgG4 level. IgG4-RD in the stomach is rare, and occasionally diagnosed as gastric subepithelial tumor (SET) by endoscopy or computed tomography scan. Two female patients in the age group of 40-50 years were diagnosed with 4 cm sized gastric SET. One underwent laparoscopic gastric wedge resection. Another one had a history of subtotal gastrectomy for early gastric cancer and idiopathic thrombocytopenic purpura with oral steroids administration...
March 2018: Journal of Gastric Cancer
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