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IgG4 related disease

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https://www.readbyqxmd.com/read/27896847/a-case-of-leucocytoclastic-vasculitis-as-a-complication-of-igg4-related-skin-disease
#1
S Nakagawa, Y Nakamura, S Yasui, O Yokosuka, H Matsue
No abstract text is available yet for this article.
November 29, 2016: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/27896569/anti-pituitary-antibodies-against-corticotrophs-in-igg4-related-hypophysitis
#2
Naoko Iwata, Shintaro Iwama, Yoshihisa Sugimura, Yoshinori Yasuda, Kohtaro Nakashima, Seiji Takeuchi, Daisuke Hagiwara, Yoshihiro Ito, Hidetaka Suga, Motomitsu Goto, Ryoichi Banno, Patrizio Caturegli, Teruhiko Koike, Yoshiharu Oshida, Hiroshi Arima
PURPOSE: IgG4-related disease is a systemic inflammatory disease characterized by infiltration of IgG4-positive plasma cells into multiple organs, including the pituitary gland. Autoimmunity is thought to be involved in the pathogenesis of IgG4-related disease. The diagnosis of IgG4-related hypophysitis (IgG4-RH) is difficult because its clinical features, such as pituitary swelling and hypopituitarism, are similar to those of other pituitary diseases, including lymphocytic hypophysitis and sellar/suprasellar tumors...
November 28, 2016: Pituitary
https://www.readbyqxmd.com/read/27891673/igg4-related-disease-experience-of-100-consecutive-cases-from-a-specialist-centre
#3
Adrian C Bateman, Emma L Culver
AIMS: To describe the features of 100 consecutive cases referred to a single UK institution, in which a diagnosis of IgG4-related disease (IgG4-RD) was under consideration. METHODS AND RESULTS: The histological features were reviewed by a single histopathologist and cases categorised using the 2012 Boston criteria: Category 1 - histologically highly suggestive of IgG4-RD; 2 - probable histopathological features of IgG4-RD; 3 - insufficient histopathological features of IgG4-RD...
November 28, 2016: Histopathology
https://www.readbyqxmd.com/read/27886799/igg4-related-disease-advances-in-the-diagnosis-and-treatment
#4
REVIEW
Pilar Brito-Zerón, Xavier Bosch, Manuel Ramos-Casals, John H Stone
IgG4-related disease is a rare immune-mediated systemic disease with the capability of involving essentially any organ. Although the presenting clinical features vary substantially according to the speciality to which patients present first, perhaps the most common clinical presentation is that of single or multiple organ enlargement, arousing suspicion of cancer. The disease is frequently diagnosed unexpectedly in pathological specimens or on imaging studies. The diagnostic approach is complex and includes not only IgG4-related tests (serum levels, circulating plasmablasts, and specific immunohistochemical studies), but also clinical, laboratory, and imaging tests as well as the typical histopathological features (lymphocytic infiltration, storiform fibrosis, eosinophilic infiltration, and obliterative phlebitis)...
April 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27884179/factors-related-to-renal-cortical-atrophy-development-after-glucocorticoid-therapy-in-igg4-related-kidney-disease-a-retrospective-multicenter-study
#5
Ichiro Mizushima, Motohisa Yamamoto, Dai Inoue, Shinichi Nishi, Yoshinori Taniguchi, Yoshifumi Ubara, Shoko Matsui, Tetsuhiko Yasuno, Hitoshi Nakashima, Hiroki Takahashi, Kazunori Yamada, Hideki Nomura, Masakazu Yamagishi, Takao Saito, Mitsuhiro Kawano
BACKGROUND: In immunoglobulin G4-related kidney disease (IgG4-RKD), focal or diffuse renal cortical atrophy is often observed in the clinical course after glucocorticoid therapy. This study aimed to clarify the factors related to renal atrophy after glucocorticoid therapy in IgG4-RKD. METHODS: We retrospectively evaluated clinical features including laboratory data and computed tomography (CT) findings before and after glucocorticoid therapy in 23 patients diagnosed with IgG4-RKD, all of whom were followed up for more than 24 months...
November 25, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/27871394/atypical-presentation-of-igg4-related-disease-of-the-eyelid
#6
Mamta Shah, Charles Shao, Roman Shinder
No abstract text is available yet for this article.
December 2016: Ophthalmology
https://www.readbyqxmd.com/read/27866757/differential-diagnosis-of-igg4-related-sialadenitis-primary-sj%C3%A3-gren-syndrome-and-chronic-obstructive-submandibular-sialadenitis
#7
X Hong, W Li, X-Y Xie, Z-Y Zhang, Y Chen, Y Gao, X Peng, J-Z Su, Y-Y Zhang, Z Wang, Z-G Cai, L Zhang, Y-Y Liu, J He, L-M Ren, Z-G Li, G-Y Yu
Our aim was to differentiate IgG4-related sialadenitis, primary Sjögren syndrome, and chronic obstructive submandibular sialadenitis by analysing clinical, radiographic, and pathological features. Fifty-five patients, 50, and 50 were enrolled, respectively and their baseline characteristics and serological, sialographic, and pathological findings compared. The male:female ratio for IgG4-related sialadenitis was 1:1.2 for primary Sjögren syndrome 1:15.7, and for chronic obstructive submandibular sialadenitis1:0...
November 17, 2016: British Journal of Oral & Maxillofacial Surgery
https://www.readbyqxmd.com/read/27864854/igg4-related-hypophysitis-is-highly-prevalent-among-cases-of-histologically-confirmed-hypophysitis
#8
Christian Bernreuther, Christopher Illies, Jörg Flitsch, Michael Buchfelder, Rolf Buslei, Markus Glatzel, Wolfgang Saeger
IgG4-related disease is an immune-mediated disease with manifestations in most organ systems among them the pituitary gland. To date, few cases of histologically confirmed cases of IgG-related hypophysitis have been reported. The aim of this study was to retrospectively determine the prevalence of IgG4-related hypophysitis among cases previously diagnosed as primary hypophysitis (lymphocytic hypophysitis, granulomatous hypophysitis, and hypophysitis not otherwise specified). Histological and immunohistochemical analysis revealed that 12 of 29 cases (41...
November 19, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27855231/association-of-newly-diagnosed-type-1-diabetes-and-autoimmune-pancreatitis
#9
Laila Ennazk, Ghizlane El Mghari, Nawal El Ansari
: Autoimmune pancreatitis is a new nosological entity in which a lymphocytic infiltration of the exocrine pancreas is involved. The concomitant onset of autoimmune pancreatitis and type 1 diabetes has been recently described suggesting a unique immune disturbance that compromises the pancreatic endocrine and exocrine functions. We report a case of type1 diabetes onset associated with an autoimmune pancreatitis in a young patient who seemed to present a type 2 autoimmune polyglandular syndrome...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27853829/-hepatopancreaticobiliary-diseases-in-igg4-associated-autoimmune-diseases
#10
L Grenacher
BACKGROUND: IgG4-related diseases are rare systemic multiorgan diseases and can thus affect any organ system. The incidence of diagnosis has significantly increased due to increasing awareness. OBJECTIVE: In the abdomen the hepatopancreaticobiliary system provides an essential organ system for the expression of IgG4-associated autoimmune diseases. The focus here is autoimmune pancreatitis type 1 but IgG4-associated sclerosing cholangitis and IgG4-associated hepatopathy, which can also occur in combination are less well-known...
November 16, 2016: Der Radiologe
https://www.readbyqxmd.com/read/27848036/the-histopathology-of-igg4-related-disease
#11
Mehmet Ozgur Avincsal, Yoh Zen
IgG4-related disease is a multi-organ immune-mediated chronic fibroinflammatory condition characterized by elevated serum IgG4 concentrations, tumefaction, and tissue infiltration by IgG4-positive plasma cells. The exact etiology of IgG4-related disease remains unclear with no known role of the IgG4 molecule itself being identified. Although the pancreas and salivary glands are the main organs affected, the involvement of other organs has also been reported. This multi-organ disease mimics a large number of malignant, infectious, and inflammatory disorders; therefore, a prompt differential diagnosis is important for selecting the right therapeutic strategy...
November 16, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27846767/a-multicenter-phase-ii-prospective-clinical-trial-of-glucocorticoid-for-patients-with-untreated-igg4-related-disease
#12
Yasufumi Masaki, Shoko Matsui, Takako Saeki, Hiroto Tsuboi, Shintaro Hirata, Yasumori Izumi, Taiichiro Miyashita, Keita Fujikawa, Hiroaki Dobashi, Kentaro Susaki, Hisanori Morimoto, Kazutaka Takagi, Mitsuhiro Kawano, Tomoki Origuchi, Yoko Wada, Naoki Takahashi, Masanobu Horikoshi, Hiroshi Ogishima, Yasunori Suzuki, Takafumi Kawanami, Haruka Iwao Kawanami, Tomoyuki Sakai, Yoshimasa Fujita, Toshihiro Fukushima, Masatoshi Saito, Ritsuro Suzuki, Yuko Morikawa, Tadashi Yoshino, Shigeo Nakamura, Masaru Kojima, Nozomu Kurose, Yasuharu Sato, Yoshiya Tanaka, Susumu Sugai, Takayuki Sumida
"Objective" Although glucocorticoids are effective for patients with IgG4-related disease, the treatment has not yet been standardized. Therefore, the treatment strategy should be established. "Patients and methods" Patients who fulfilled the comprehensive diagnostic criteria for definite IgG4-related disease were started on prednisolone (0.6mg/kg body weight) with the dose reduced every 2 weeks. The subsequent maintenance dose and need for prednisolone were determined for individual patients. The primary endpoint was the complete remission (CR) rate at 1 year...
November 16, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27846747/a-case-of-alopecia-as-igg4-related-skin-disease
#13
Takaharu Ikeda, Chikako Kaminaka, Fukumi Furukawa
A 53-year-old man with autoimmune pancreatitis as IgG4-related disease presented alopecia. Immunostaining showed perifollicular infiltration of IgG4-positive cells and perifollicular/intrafollicular infiltration of predominantly CD4-positive cells especially in the upper and lower parts. Alopecia areata is characterized by perifollicular/intrafollicular lymphocyte infiltration of the lower bulb and inflammation in the upper dermis. We determined the patient had IgG4-related skin disease, rather than alopecia areata...
November 16, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27843911/immunoglobulin-g4-related-ophthalmic-disease-involving-the-sclera-misdiagnosed-as-intraocular-tumor-report-of-one-case
#14
Hiroshi Goto, Shunichiro Ueda
AIMS: Immunoglobulin G4 (IgG4)-related disease is known to have diverse clinical manifestations. We report a case of IgG4-related ophthalmic disease misdiagnosed as intraocular tumor. METHODS: Case report. RESULTS: A 41-year-old man was referred to our department with a diagnosis of intraocular tumor, presumably choroidal melanoma. Although MR imaging was compatible with choroidal melanoma, he had been observed periodically without treatment because fundus findings were not typical of melanoma and visual function was preserved...
October 2016: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/27843427/isolated-liver-hilar-infiltration-by-igg4-inflammation-mimicking-cholangiocarcinoma
#15
Laurent Bochatay, Pietro Majno, Emiliano Giostra, Jean Louis Frossard
IgG4-related disease represents a heterogeneous group of disease characterized by infiltration of various tissues by IgG4 plasmocytes. In case of liver infiltration, this condition classically mimics primary sclerosing cholangitis or multifocal cholangiocarcinoma due to inflammation that preferentially affects the intra- and extrahepatic bile duct. Diagnostic criteria have recently been reviewed in order to better define the disease and help physicians make the diagnosis. Herein, we present the case of a patient who died after liver surgery for suspected cholangiocarcinoma that finally turned out to be IgG4-associated liver disease, a condition being out of current consensual criteria...
September 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27832379/immunogenetics-of-igg4-related-aip
#16
Masao Ota, Takeji Umemura, Shigeyuki Kawa
Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis characterized by high serum IgG4 concentration and a variety of complicating extra-pancreatic lesions. AIP has the features of a complex disease that is caused by multifactorial genes. However, the genetic factors underlying AIP have not been elucidated conclusively. Association studies by the candidate-gene approach and genome-wide association studies (GWAS) have revealed several susceptibility genes for AIP, including HLA DRB1*04:05-DQB1*04:01, FCRL3, CTLA4, and KCNA3, albeit in small-scale analyses...
November 11, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27832299/-immunological-principles-of-igg4-related-diseases
#17
T Witte
In contrast to the other IgG subclasses, IgG4 does not bind to low affinity Fc receptors or activate the classical complement pathway. In addition, it is unstable and can dissociate into two hemimolecules; therefore, IgG4 most likely has an immunosuppressive role. On the other hand, there a few examples of an immunostimulatory role of IgG4 antibodies; therefore, the function of IgG4 in IgG4 related diseases is not yet entirely clear. The trigger factors of IgG4 related diseases (allergic or autoimmune) are still under debate...
November 10, 2016: Der Radiologe
https://www.readbyqxmd.com/read/27831514/diagnostic-challenges-in-a-case-of-igg4-rd-affecting-the-temporal-bone
#18
Jackson Ross Vuncannon, Nicholas John Panella, Kelly R Magliocca, Douglas E Mattox
INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is a recently described fibroinflammatory condition with a characteristic histology. While IgG4-RD can affect a great variety of anatomical sites, it has been seldom described in the temporal bone. METHODS: Herein, a case IgG4-RD occurring in the temporal bone of a 35-year-old woman is reported. DISCUSSION: This case of IgG4-RD of the temporal bone proved a uniquely challenging diagnosis due to slightly atypical histology falling outside of "highly suggestive" criteria...
November 9, 2016: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/27830722/comprehensive-polymerase-chain-reaction-assay-for-detection-of-pathogenic-dna-in-lymphoproliferative-disorders-of-the-ocular-adnexa
#19
Yoshihiko Usui, Narsing A Rao, Hiroshi Takase, Kinya Tsubota, Kazuhiko Umazume, Daniel Diaz-Aguilar, Takeshi Kezuka, Manabu Mochizuki, Hiroshi Goto, Sunao Sugita
Infectious agents have been identified as a major cause of specific types of human cancers worldwide. Several microorganisms have been identified as potential aggravators of ocular adnexal neoplasms; however, given the rarity of these neoplasms, large epidemiological studies are difficult to coordinate. This study aimed to conduct an exhaustive search for pathogenic DNA in lymphoproliferative disorders (LPD) of the ocular adnexa in a total of 70 patients who were diagnosed with LPD of the ocular adnexa between 2008 and 2013...
November 10, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27828690/immunoglobulin-g4-related-thyroid-disorders-diagnostic-challenges-and-clinical-outcomes
#20
Deep Dutta, Arvind Ahuja, Chitra Selvan
Ig-G4 related disease (IgG4RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the start of this century only. It is currently one of the hottest areas of clinical and translational research across specialties. Thyroid involvement in IgG4RD is rare, believed to occur in less than 4% of cases, may be isolated or may be associated with other organ involvement. As of today Riedel's thyroiditis, fibrosing variant of Hashimoto's thyroiditis, and few patients of Graves' orbitopathy represent the types of IgG4-related thyroid disease (IgG4RTD)...
2016: Endokrynologia Polska
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