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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/28079853/solely-lung-involved-igg4-related-disease-a-case-report-and-review-of-the-literature
#1
Xiao-Qin Zhang, Guo-Ping Chen, Sheng-Chang Wu, Sa Yu, Hong Wang, Xuan-Yi Chen, Zhuo-Chao Ren
By analyzing the clinical data of 1 case of IgG4-related lung disease(IgG4-RLD) and the review of literature, the author investigated the clinical characteristics of IgG4-RLD. IgG4-RLD is a rare disease characterized by significant elevation of serum IgG4 and infiltration of a large number of IgG4+ plasma cells. The clinical manifestations of the disease were nonspecific, and the imaging features were mixed with several types. The disease can only be involved in the lung, but also multiple organ involvement...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28070877/idiopathic-retroperitoneal-fibrosis-and-its-overlap-with-igg4-related-disease
#2
REVIEW
Giovanni Maria Rossi, Rossana Rocco, Eugenia Accorsi Buttini, Chiara Marvisi, Augusto Vaglio
Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis...
January 9, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28049999/overlap-of-post-obstructive-diuresis-and-unmasked-diabetes-insipidus-in-a-case-of-igg4-related-retroperitoneal-fibrosis-and-tuberoinfundibular-hypophysitis-a-case-report-and-review-of-the-literature
#3
Midori Sasaki Yatabe, Kimio Watanabe, Yoshimitsu Hayashi, Junichi Yatabe, Satoshi Morimoto, Atsuhiro Ichihara, Masaaki Nakayama, Tsuyoshi Watanabe
The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28005278/igg4-related-disease-and-lymphocyte-variant-hypereosinophilic-syndrome-a-comparative-case-series
#4
Mollie N Carruthers, Sujin Park, Graham W Slack, Bakul I Dalal, Brian F Skinnider, David F Schaeffer, Jan P Dutz, Joanna K Law, Fergal Donnellan, Vladimir Marquez, Michael Seidman, Patrick C Wong, Andre Mattman, Luke Y C Chen
OBJECTIVE: To compare the clinical and laboratory features of IgG4-related disease (IgG4-RD) and lymphocyte variant-hypereosinophilic syndrome (L-HES), two rare diseases that often present with lymphadenopathy, gastrointestinal symptoms, eosinophilia and elevated immunoglobulins/IgE. METHOD: Comparative case series of 31 IgG4-RD and 13 L-HES patients. RESULTS: Peripheral blood eosinophilia was present in 8/31 IgG4-RD patients compared to 13/13 L-HES patients (median eosinophils 0...
December 22, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27994271/retroperitoneal-fibrosis-the-state-of-the-art
#5
REVIEW
Marta Runowska, Dominik Majewski, Mariusz Puszczewicz
Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis - IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD)...
2016: Reumatologia
https://www.readbyqxmd.com/read/27989497/dacryoadenitis-associated-with-igg4-related-disease-in-an-afro-colombian-adolescent
#6
Gabriel Sebastián Díaz-Ramírez, Luis Fernando Medina-Quintero, Alexander Salinas-César, Andrés Felipe Zea-Vera
IgG4-related disease (IgG4-RD) is a recently recognized clinical condition with multiple aspects not yet elucidated. It is characterized by a fibrous inflammatory process that involves multiple organs and clinical, serological and histopathological findings, which represent a major challenge for the clinician. Classically described as an expansive tumor lesion with storiform fibrosis, lymphoplasmacytic infiltration (IgG4-positive) and elevated serum IgG4. Clinical features are variable, and pancreatic as well as extrapancreatic involvement has been reported, more frequently in Asian men over 50 years and rarely described in black people...
December 15, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27988433/igg4-related-disease-evidence-from-six-recent-cohorts
#7
REVIEW
Fernando Martínez-Valle, Andreu Fernández-Codina, Iago Pinal-Fernández, Olimpia Orozco-Gálvez, Miquel Vilardell-Tarrés
IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosing disorder. In this review we aim to describe and compare the characteristics of the six largest IgG4-RD cohorts, since the new 2012 consensus diagnostic criteria were released. These observational studies were published between 2012 and 2015. Patients were included using the comprehensive diagnostic criteria or the 2012 consensus criteria. Results were reviewed and summarized. Most patients were middle aged men. Fibro-inflammatory masses developed in virtually all organs except the brain, with an unexplained preference for salivary glands, lymph nodes and pancreas...
December 15, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27956101/recent-advances-in-knowledge-regarding-the-head-and-neck-manifestations-of-igg4-related-disease
#8
REVIEW
Kenichi Takano, Motohisa Yamamoto, Hiroki Takahashi, Tetsuo Himi
IgG4-related disease (IgG4-RD) is a chronic inflammatory disorder, characterized by elevated serum IgG4 levels as well as abundant infiltration of IgG4-positive plasmacytes and fibrosis in various organs, including the head and neck region. In particular, the salivary glands, orbit, and thyroid are common sites of disease involvement. IgG4-RD is diagnosed based on various clinical, serological, and histopathological findings, none of which are pathognomonic. Hence, various differential diagnoses, which exhibit elevated serum IgG4 levels and infiltration of IgG4-postive cells into tissues, need to be excluded, especially malignant diseases and mimicking disorders...
December 9, 2016: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/27891673/igg4-related-disease-experience-of-100-consecutive-cases-from-a-specialist-centre
#9
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/27846767/a-multicenter-phase-ii-prospective-clinical-trial-of-glucocorticoid-for-patients-with-untreated-igg4-related-disease
#10
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 Kawanami Iwao, 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 (0.6 mg/kg body weight) with the dose reduced every two weeks. The subsequent maintenance dose and need for prednisolone were determined for individual patients...
December 15, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27831514/diagnostic-challenges-in-a-case-of-igg4-rd-affecting-the-temporal-bone
#11
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/27817178/roles-of-regulatory-t-and-b-cells-in-igg4-related-disease
#12
Kazushige Uchida, Kazuichi Okazaki
Immunoglobulin G4 (IgG4) -related disease (RD) is a newly recognized systemic disease. Although there are several forms of IgG4-RD reported under various names, depending on the target organ and characteristics, patients with IgG4-RD manifest several immunologic and histologic abnormalities including increased levels of serum IgG4 and storiform fibrosis with infiltration of lymphocytes and IgG4-positive plasmacytes in the involved organs. However, the pathophysiology remains unclear. Regulatory immune cells play an important role in several immune-related diseases...
November 6, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27801343/-a-retrospective-case-control-study-of-immunoglobulin-g4-related-disease-combined-with-malignancy
#13
Y L Feng, D Wu, S Y Zhang, X Wu, H J Shu, A M Yang, J M Qian
IgG4-related disease (IgG4-RD) has been proved to be associated with malignancy.The incidence and risk factors of malignancy development in IgG4-RD were not clear. Nine IgG4-RD patients with malignancies and 27 IgG4-RD control cases were analyzed for risk factors and clinical features. The incidence of malignancy in IgG4-RD was 3.3%, higher than age-controlled general population. Smoking history was significantly more common in patients with malignancies than in the control group (9/9 vs 16/27, P<0.05). A total of 6/9 malignancies occurred within the first year after the diagnosis of IgG4-RD...
November 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27799828/serum-total-igg-and-igg4-levels-in-thyroid-eye-disease
#14
Aileen Sy, Rona Z Silkiss
PURPOSE: To investigate the relationship between immunoglobulin G (IgG)4-related disease (IgG4-RD) and thyroid eye disease (TED) with respect to IgG levels. PATIENTS AND METHODS: A retrospective review of total IgG, IgG subclass, and thyroid stimulating immunoglobulin (TSI) levels in 24 patients with TED. RESULTS: Five patients (20.8%) demonstrated serum IgG4 levels consistent with IgG4-RD without any additional systemic disease. Total IgG and IgG subclass levels were found to be an inadequate proxy for TSI elevation...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27797069/igg4-related-sclerosing-mesenteritis-causing-bowel-obstruction-a-case-report
#15
Atsushi Abe, Tatsuya Manabe, Nobuyoshi Takizawa, Takashi Ueki, Daisuke Yamada, Kinuko Nagayoshi, Yoshihiko Sadakari, Hayato Fujita, Shuntaro Nagai, Hidetaka Yamamoto, Yoshinao Oda, Masafumi Nakamura
Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease primarily involving the small-bowel mesentery. Recently, SM was reported to be closely related to IgG4-related disease (IgG4-RD). This report describes a patient with SM associated with IgG4-RD. A 77-year-old woman with a history of surgery for ectopic pregnancy and wound dehiscence presented with intestinal obstruction. Abdominal enhanced computed tomography (CT) revealed an enhanced, radially shaped, oval mass, 3 cm in diameter, with an unclear rim in the mesentery of the distal ileum, which may have involved the distal ileum...
December 2016: Surgical Case Reports
https://www.readbyqxmd.com/read/27785920/a-case-of-igg4-related-tubulointerstitial-nephritis-and-membranous-glomerulonephritis-during-the-clinical-course-of-gastric-cancer-imaging-features-of-igg4-related-kidney-disease
#16
Shigeto Horita, Hiroshi Fujii, Ichiro Mizushima, Yuhei Fujisawa, Satoshi Hara, Kazunori Yamada, Dai Inoue, Kenichi Nakajima, Kenichi Harada, Mitsuhiro Kawano
We describe an 81-year-old man with immunoglobulin G4-related disease (IgG4-RD) presenting with submandibular gland, lymph node, lung, kidney, aortic wall, and prostate lesions with concomitant gastric cancer. After curative surgical treatment of the gastric cancer, corticosteroid therapy for progressively decreasing renal function was started. Before starting steroid therapy, fluorodeoxyglucose positron emission tomography-computed tomography revealed multiple lesions of IgG4-RD but no metastasis of the cancer...
October 27, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27766198/igg4-related-lung-disease-extending-to-the-thoracic-vertebrae
#17
Alexandros Stamatopoulos, Davide Patrini, Efstratios Koletsis, Elaine Borg, Reena Khiroya, Martin Hayward, David Lawrence, Nikolaos Panagiotopoulos
IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect practically every organ. Although it was first identified in pancreas and salivary glands, major organs like liver, biliary tree, kidney, thyroid glands and lungs are commonly involved, sometimes resulting in organ failure. We describe a case of an 41-year-old man presented with back pain after a rotator cuff injury. A Computed Tomography (CT) revealed incidentally a right lower lobe paravertebral lesion extending across the T5 and T6 vertebral levels and invading into the adjacent pleural surface...
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27744510/th1-th2-immune-balance-and-other-t-helper-subsets-in-igg4-related-disease
#18
Masafumi Moriyama, Seiji Nakamura
IgG4-related disease (IgG4-RD) is a systemic disease characterized by elevated serum IgG4 levels and a strong infiltration of IgG4-positive plasma cells in various organs. IgG4-RD patients also frequently suffer from allergic diseases, including asthma and atopic dermatitis. It is well known that T helper type 2 (Th2) cells have an important role in the initiation of allergic diseases, and Th2 cytokines such as interleukin (IL)-4 and IL-13 promote class switching to IgG4. Therefore, IgG4-RD is considered to be a Th2-predominant disease...
October 16, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27744509/igg4-related-disease-and-innate-immunity
#19
Tomohiro Watanabe, Kouhei Yamashita, Masatoshi Kudo
An increased number of clinicopathological studies on autoimmune pancreatitis, cholangitis, and sialoadenitis have led to the recognition of immunoglobulin G4-related disease (IgG4-RD) as a novel disorder, characterized by elevated levels of serum IgG4 and infiltration of IgG4-expressing plasma cells in the affected organs. Although the immunological background associated with the development of IgG4-RD remains poorly understood, recent studies have suggested involvement of the innate immune response in its pathogenesis...
October 16, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27729479/igg4-related-disease-of-bilateral-temporal-bones
#20
Lilun Li, Bryan Ward, Margaret Cocks, Amir Kheradmand, Howard W Francis
OBJECTIVE: IgG4-related disease (IgG4-RD) is an idiopathic inflammatory condition that causes pseudotumor formation in single or multiple organs, including those of the head and neck. Temporal bone involvement is rare, with only 3 cases of unilateral temporal bone IgG4-RD described in the literature. We report the first known case of IgG4-RD of bilateral temporal bones and describe its clinical presentation, diagnosis, and treatment. METHODS: The patient was a 52-year-old man with latent tuberculosis (TB) who presented with a 10-year history of bilateral profound hearing loss and vestibular dysfunction...
October 10, 2016: Annals of Otology, Rhinology, and Laryngology
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