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"IgG4-related disease"

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https://www.readbyqxmd.com/read/28238541/igg4-related-sclerosing-cholangitis-overlapping-with-autoimmune-hepatitis-report-of-a-case
#1
Hongyan Li, Li Sun, David R Brigstock, Lina Qi, Runping Gao
BACKGROUND: IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-related disease (IgG4-RD) but the presence of IgG4-SC in the porta hepatis is difficult to differentiate from hilar cholangiocarcinoma (HCCA). IgG4-related autoimmune hepatitis (IgG4-related AIH) is extremely rare and it is not fully clear whether IgG4-related AIH is a hepatic manifestation of IgG4-RD or a subtype of AIH. CASE PRESENTATION: We present a rare case of a 52-year-old male who was admitted with obstructive jaundice and itchy skin...
February 10, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28228909/igg4-related-disease-in-an-adolescent-with-radiologic-pathologic-correlation
#2
Renato Cesar Ferreira da Silva, Scott M Lieberman, Henry T Hoffman, Bruno Policeni, Amani Bashir, Richard J H Smith, T Shawn Sato
Immunoglobulin G4-related disease (IgG4RD) is an immune-mediated condition characterized by lymphoplasmacytic infiltrates and fibrosis of affected organs. IgG4RD may affect many different organs either individually or together in a multiorgan condition and, thus, incorporates a wide range of fibroinflammatory phenotypes with shared pathologic features. Although IgG4RD most commonly occurs in late adulthood, it may affect children and adolescents. Only one case of IgG4RD presenting as isolated submandibular gland involvement has been reported in the pediatric population...
March 2017: Radiology case reports
https://www.readbyqxmd.com/read/28223204/increases-in-ige-eosinophils-and-mast-cells-can-be-used-in-diagnosis-and-to-predict-relapse-of-igg4-related-disease
#3
Emma L Culver, Ross Sadler, Adrian C Bateman, Mateusz Makuch, Tamsin Cargill, Berne Ferry, Rob Aalberse, Eleanor Barnes, Theo Rispens
BACKGROUND & AIMS: Immunoglobulin G4-related disease (IgG4-RD) is characterized by increased serum levels of IgG4 and infiltration of biliary, and pancreatic, and other tissues by IgG4-positive plasma cells. We assessed the prevalence of allergy and/or atopy, serum and tissue IgE antibodies, and blood and tissue eosinophils in patients with IgG4-RD. We investigated the association between serum IgE and diagnosis and relapse of this disease. METHODS: We performed a prospective study of 48 patients with IgG4-RD, 42 patients with an increased serum level of IgG4 with other inflammatory and autoimmune conditions (disease controls), and 51 healthy individuals (healthy controls) recruited from Oxford, United Kingdom from March 2010 through March 2014, and followed for a median of 41 months (range 3-73 months)...
February 18, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28222449/characterization-of-cutaneous-plasmacytosis-at-different-disease-stages
#4
Pa-Fan Hsiao, Yu-Hung Wu
BACKGROUND/AIMS: Cutaneous plasmacytosis is rare and still not well understood. A retrospective study was made of 9 Chinese patients with 1- to 15-year histories of biopsy-proven cutaneous plasmacytosis diagnosed between 2003 and 2015. METHODS: Patient records and archival photographs helped establish the pattern and duration of skin lesions, and skin biopsy specimens provided additional data. RESULTS: The mean age at diagnosis was 46.4 years...
February 22, 2017: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/28219556/igg4-related-disease-causing-rapid-evolution-of-a-severe-aortic-valvular-stenosis
#5
Samuel Bruls, Audrey Courtois, Philippe Delvenne, Roland Hustinx, Michel Moutschen, Laurence De Leval, Jean-Olivier Defraigne, Natzi Sakalihasan
Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recognized emerging entity characterized by chronic fibroinflammation that can affect every organ but rarely affects the cardiovascular system. We report a rare case of IgG4-RSD involving an aortic valve that resulted in rapid progression of an aortic valvular stenosis and was successfully treated by aortic valve replacement and corticosteroids.
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28207938/comparison-of-clinical-and-pathological-features-of-lung-lesions-of-systemic-igg4-related-disease-and-idiopathic-multicentric-castleman-s-disease
#6
Yasuhiro Terasaki, Shinichro Ikushima, Shoko Matsui, Akira Hebisawa, Yasunori Ichimura, Shinyu Izumi, Masuo Ujita, Machiko Arita, Keisuke Tomii, Youko Komase, Isoko Owan, Tetsuji Kawamura, Yasuo Matsuzawa, Miho Murakami, Hiroshi Ishimoto, Hiroshi Kimura, Masashi Bando, Norihiro Nishimoto, Yoshinori Kawabata, Yuh Fukuda, Takashi Ogura
AIMS: The lung lesion (IgG4-L) of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion (iMCD-L) of idiopathic multicentric Castleman's disease (iMCD). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data. METHODS AND RESULTS: Nine patients had IgG4-L (high levels of serum IgG4 and of IgG4(+) cells in lung specimens; typical extrapulmonary manifestations)...
February 16, 2017: Histopathology
https://www.readbyqxmd.com/read/28205524/interleukin-33-produced-by-m2-macrophages-and-other-immune-cells-contributes-to-th2-immune-reaction-of-igg4-related-disease
#7
Sachiko Furukawa, Masafumi Moriyama, Kensuke Miyake, Hitoshi Nakashima, Akihiko Tanaka, Takashi Maehara, Mana Iizuka-Koga, Hiroto Tsuboi, Jun-Nosuke Hayashida, Noriko Ishiguro, Masaki Yamauchi, Takayuki Sumida, Seiji Nakamura
IgG4-related disease (IgG4-RD) is characterized by elevated serum IgG4 and marked infiltration of IgG4-positive cells in multiple organs. Interleukin-33 (IL-33) is a recently described cytokine that is secreted by damaged epithelial cells, macrophages, and dendritic cells, and potently activates helper T type 2 (Th2) immune responses, which have been suggested to play a major role in IgG4 production of IgG4-RD. Here, we assessed the expression of IL-33 and related molecules in the salivary glands (SGs) of patients with IgG4-RD versus that in patients with Sjögren's syndrome (SS) and controls...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28202062/isolated-igg4-related-sclerosing-cholangitis-misdiagnosed-as-malignancy-in-an-area-with-endemic-cholangiocarcinoma-a-case-report
#8
Narongsak Rungsakulkij, Pattana Sornmayura, Penampai Tannaphai
BACKGROUND: The most common cause of perihilar obstruction is cholangiocarcinoma, especially in Thailand. Benign perihilar stricture represents less than 20% of cases. IgG4-related disease and IgG4-related sclerosing cholangitis, however, have been receiving increased recognition. Isolated IgG4-related sclerosing cholangitis is less common. The preoperative diagnosis of IgG4-related sclerosing cholangitis without pancreatic involvement is very difficult because the clinical presentation and preoperative evaluation are extremely difficult to distinguish from perihilar cholangiocarcinoma...
February 15, 2017: BMC Surgery
https://www.readbyqxmd.com/read/28197739/diagnosis-and-treatment-of-igg4-related-disease
#9
Terumi Kamisawa, Kazuichi Okazaki
It is critical to differentiate IgG4-related disease (IgG4-RD) from malignant tumor and similar disease of the affected organ to apply appropriate therapy and avoid unnecessary surgery. IgG4-RD is diagnosed on combination of typical radiological findings; elevation of serum IgG4 levels; histopathological findings of abundant infiltration of IgG4-positive plasma cells and lymphocytes, storiform fibrosis , and obliterative phlebitis ; association with other IgG4-related diseases; and response to steroids. Histopathological approach is particularly recommended...
February 15, 2017: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/28183334/circulating-plasmablasts-plasma-cells-a-potential-biomarker-for-igg4-related-disease
#10
Wei Lin, Panpan Zhang, Hua Chen, Yu Chen, Hongxian Yang, Wenjie Zheng, Xuan Zhang, Fengxiao Zhang, Wen Zhang, Peter E Lipsky
BACKGROUND: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a multisystem fibroinflammatory disease. We previously reported that a circulating cell population expressing CD19(+)CD24(-)CD38(hi) was increased in patients with IgG4-RD. In this study, we aimed to document that this cell population represented circulating plasmablasts/plasma cells, to identify the detailed phenotype and gene expression profile of these IgG4-secreting plasmablasts/plasma cells, and to determine whether this B-cell lineage subset could be a biomarker in IgG4-related disease (IgG4-RD)...
February 10, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28166682/clonally-expanded-cytotoxic-cd4-t-cells-and-the-pathogenesis-of-igg4-related-disease
#11
Hamid Mattoo, John H Stone, Shiv Pillai
IgG4-related disease (IgG4-RD) is a systemic condition of unknown cause characterized by highly fibrotic lesions, with dense lymphoplasmacytic infiltrates containing a preponderance of IgG4-expressing plasma cells. CD4(+) T cells and B cells constitute the major inflammatory cell populations in IgG4-RD lesions. IgG4-RD patients with active, untreated disease show a marked expansion of plasmablasts in the circulation. Although the therapeutic depletion of B cells suggests a role for these cells in the disease, a direct role for B cells or IgG4 in the pathogenesis of IgG4-RD is yet to be demonstrated...
February 2017: Autoimmunity
https://www.readbyqxmd.com/read/28165852/current-approach-to-the-diagnosis-of-igg4-related-disease-combination-of-comprehensive-diagnostic-and-organ-specific-criteria
#12
Hisanori Umehara, Kazuichi Okazaki, Takuji Nakamura, Tomomi Satoh-Nakamura, Akio Nakajima, Mitsuhiro Kawano, Tsuneyo Mimori, Tsutomu Chiba
IgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criteria has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan, published comprehensive diagnostic criteria for IgG4-RD...
February 6, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28158996/igg4-positive-cell-infiltration-in-various-cardiovascular-disorders-results-from-histopathological-analysis-of-surgical-samples
#13
Ryoto Hourai, Satomi Kasashima, Koichi Sohmiya, Yohei Yamauchi, Hideki Ozawa, Yoshinobu Hirose, Yasuhiro Ogino, Takahiro Katsumata, Masahiro Daimon, Shu-Ichi Fujita, Masaaki Hoshiga, Nobukazu Ishizaka
BACKGROUND: The diagnosis of Immunoglobulin G4 (IgG4)-related disease (IgG4-RD), in general, depends on serum IgG4 concentrations and histopathological findings; therefore, diagnosis of IgG4-RD in cardiovascular organs/tissues is often difficult owing to the risk of tissue sampling. METHODS: Prevalence of IgG4-positive lymphoplasmacytic infiltration in 103 consecutive cardiovascular surgical samples from 98 patients with various cardiovascular diseases was analyzed immunohistochemically...
February 3, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28149653/igg4-related-disease-presenting-as-isolated-scleritis
#14
Eran Berkowitz, Ella Arnon, Alona Yaakobi, Yuval Cohen, Beatrice Tiosano
A rare case of IgG4-related disease (IgG4-RD) manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms...
2017: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/28134406/-the-ormond-s-disease-an-intriguing-obstructive-nephrouropathy
#15
Rocco Manganelli, Serena Manganelli, Salvatore Iannaccone, Walter De Simone
Idiopathic retroperitoneal fibrosis also known as Ormonds disease is a rare disorder characterized by the development of fibrotic tissue in the retroperitoneum involving the abdominal aorta and iliac arteries, ureters and the inferior vena cava. The aberrant tissue may compress ureters leading to obstructive nephrouropathy and renal failure, which are the most common clinical manifestations of this condition. The nephrologist is often consulted to make differential diagnosis for acute renal failure and obstructive uropathy...
November 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28124667/igg4-related-disease-a-disease-we-probably-often-overlook
#16
I M Celis, R L Kriekaart, R P Aliredjo, E G van Lochem, M J D L van der Vorst, R Hassing
IgG4-related disease (IgG4-RD) is an increasingly recognised entity characterised by tumefied lesions that can affect multiple organs. Awareness of IgG4-RD is important, as it has been shown to mimic other diseases and may result in irreversible organ damage if not treated. If suspected, immunostaining for IgG4-positive plasma cells is essential for diagnosis and revision of old biopsies may be necessary.
January 2017: Netherlands Journal of Medicine
https://www.readbyqxmd.com/read/28114468/igg4-related-disease-in-monozygotic-twins-a-case-report
#17
Aurélie Grados, Thibaud Vaysse, Mikael Ebbo, Franck Carbonnel, Nicolas Schleinitz
No abstract text is available yet for this article.
January 17, 2017: Annals of Internal Medicine
https://www.readbyqxmd.com/read/28109714/biliary-tumors-with-pancreatic-counterparts
#18
Yasuni Nakanuma, Yoshiko Sudo
Some biliary diseases mimic pancreatic diseases pathologically as well as pathogenetically. Such diseases can be called "biliary diseases with pancreatic counterparts". Biliary intraepithelial neoplasm (BilIN), intraductal papillary neoplasm of bile ducts (IPNB), hepatobiliary mucinous cystic neoplasm (hMCN), and IgG4-inflammatory pseudotumor represent the biliary counterparts of pancreatic intraepithelial neoplasm (PanIN), intraductal papillary mucinous neoplasm of pancreas (IPMN), pancreatic MCN, and mass forming type 1 autoimmune pancreatitis (AIP), respectively...
December 20, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28107092/a-solid-pseudopapillary-neoplasm-of-the-pancreas-associated-with-igg4-related-pancreatitis
#19
Yuichiro Hatano, Keisuke Kawashima, Takuji Iwashita, Masaki Kimura, Masahito Shimizu, Akira Hara
A solid and cystic pancreatic lesion was incidentally found in a 62-year-old woman on abdominal computed tomography. The lesion was diagnosed as a solid pseudopapillary neoplasm by using endoscopic ultrasound-guided fine needle aspiration, and the tumor was resected. Histologically, the tumor cells had relatively small, round nuclei and papillary-like architecture in a hemorrhagic background. On immunohistochemical examination, the tumor cells were diffusely positive for nuclear β-catenin and cytoplasmic CD10...
November 1, 2016: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28105551/elevated-serum-igg4-levels-in-diagnosis-and-treatment-response-in-patients-with-idiopathic-retroperitoneal-fibrosis
#20
L G Pelkmans, T R Hendriksz, P J Westenend, H J Vermeer, E F H van Bommel
Idiopathic retroperitoneal fibrosis (iRPF) may be a manifestation of IgG4-related disease. Measuring serum IgG4 (sIgG4) may be of value in monitoring iRPF, but this has scarcely been evaluated. It is unknown if tamoxifen (TMX) affects sIgG4 levels. We performed a prospective inception cohort study of 59 patients with untreated (re)active iRPF stratified by elevated (>1.4 g/L) or normal sIgG4 level. Changes in sIgG4 levels following TMX initiation and, if treatment failed, during subsequent corticosteroid (CS) treatment were analyzed...
January 19, 2017: Clinical Rheumatology
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