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IgG4 disease

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https://www.readbyqxmd.com/read/28449468/pulmonary-inflammatory-myofibroblastic-tumor-versus-igg4-related-inflammatory-pseudotumor-differential-diagnosis-based-on-a-case-series
#1
Longfei Zhu, Jian Li, Chengwu Liu, Wenshuang Ding, Feng Lin, Chenglin Guo, Lunxu Liu
BACKGROUND: Pulmonary inflammatory myofibroblastic tumor (IMT) has been considered as a synonym for inflammatory pseudotumor (IPT) for a long time. Recent studies have indicated that IMT and IgG4-related IPT are distinct diseases. However, no consensus criteria have been recommended. Here we propose a set of criteria for the differential diagnosis. METHODS: Twenty-six archived IMT and IgG4-related IPT samples were examined for histological characteristics and the expression of IgG, IgG4, SMA and ALK-1...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28449462/pulmonary-function-tests-findings-and-their-diagnostic-value-in-patients-with-igg4-related-disease
#2
Lu Cao, Yi-Bing Chen, Da-Hui Zhao, Wen-Fang Shi, Song Meng, Li-Xin Xie
BACKGROUND: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can affect most organs. To date, there have been no detailed assessments of pulmonary function in patients with IgG4-RD. In this study, we investigated pulmonary function in IgG4-RD patients and evaluated the value of pulmonary function tests (PFTs) in diagnosing IgG4-related respiratory disease (IgG4-RRD). METHODS: This was a retrospective study of 17 patients with IgG4-RD...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28446143/igg4-related-membranous-glomerulonephritis-and-generalized-lymphadenopathy-without-pancreatitis-a-case-report
#3
Justine Huart, Stéphanie Grosch, Christophe Bovy, Michel Moutschen, Jean-Marie Krzesinski
BACKGROUND: IgG4-related disease is a recently described pathologic entity. This is the case of a patient with nephrotic syndrome and lymphadenopathy due to IgG4-related disease. Such a kidney involvement is quite peculiar and has only been described a few times recently. Renal biopsy showed a glomerular involvement with membranous glomerulonephritis in association with a tubulo-interstitial nephropathy. Moreover, the patient was not suffering from pancreatitis. CASE PRESENTATION: The patient is a middle-aged man of Moroccan origin...
April 26, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28443233/a-case-of-immunoglobulin-g4-related-respiratory-disease-with-multiple-lung-cysts-a-case-report
#4
Hironori Mikumo, Naoki Hamada, Eiji Harada, Toyoshi Yanagihara, Saiko Ogata, Hidetake Yabuuchi, Kayo Ijichi, Koichi Takayama, Yoichi Nakanishi
A 48-year-old man was admitted for evaluation of abnormal shadows on chest radiograph. Chest computed tomography (CT) showed cysts, nodules, and cervical and axillary lymphadenopathies. Elevated serum levels of IgG4 and interleukin (IL)-6 suggested IgG4-related disease (IgG4-RD) or multicentric Castleman's disease (MCD). Histologic findings of the cervical lymph node and right lung S(6) biopsies revealed numerous IgG4-positive plasma cells. Although CT findings of the lungs were atypical for IgG4-RD, consistent histologic findings, clinical symptoms, and laboratory data made us conclude IgG4-RD...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28439907/igg4-related-disease-with-bone-marrow-involvement-mimicking-multiple-myeloma
#5
Shih-Hao Tang, Ming-Hui Lin, Jeng-Shiun Du, Yi-Chang Liu, Hui-Hua Hsiao, Ta-Chih Liu
No abstract text is available yet for this article.
April 25, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28438752/igg4-related-disease-presenting-as-posterior-scleritis-and-vitritis-progressing-to-multifocal-orbital-involvement
#6
Gavin L Reynolds, Jonathan H Norris, Sher Aslam, Srilakshmi Sharma
IgG4-related disease (IgG4-RD) is a rare, chronic inflammatory condition that may involve nearly every organ system. Originally identified as a cause of autoimmune pancreatitis, its characteristic histological and clinical features have been found in a wide variety of inflammatory presentations, including the eye and orbit. Here we describe an example of a case of IgG4-RD initially presenting as scleritis and vitritis, with further progression to multifocal bilateral orbital involvement. Tissue biopsy of an orbital mass was highly characteristic of IgG4-RD histology and a rapid clinical response to corticosteroids was observed...
April 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28434701/upregulated-interleukins-il-6-il-10-and-il-13-in-immunoglobulin-g4-related-aortic-aneurysm-patients
#7
Satomi Kasashima, Atsuhiro Kawashima, Yoh Zen, Satoru Ozaki, Fuminori Kasashima, Masamitsu Endo, Yasushi Matsumoto, Kengo Kawakami
OBJECTIVE: Immunoglobulin (Ig) G4-related aortic aneurysms (IgG4-AAs) are a special aortic aneurysm among IgG4-related diseases (IgG4-RDs), which are inflammatory and fibrous conditions characterized by tumorous swelling of affected organs and high serum IgG4 concentrations. Recently, IgG4-RD pathogenesis was shown to be associated with T-helper-2 (Th2) and regulatory T (Treg) dominant cytokine production, such as interleukin (IL)-4, IL-10, and IL-13. IL-6 is a key proinflammatory cytokine contributing to lymphocyte and plasmacyte maturation and to atherosclerosis and aneurysm development...
April 20, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28428630/hinge-deleted-igg4-blocker-therapy-for-acetylcholine-receptor-myasthenia-gravis-in-rhesus-monkeys
#8
Mario Losen, Aran F Labrijn, Vivianne H van Kranen-Mastenbroek, Maarten L Janmaat, Krista G Haanstra, Frank J Beurskens, Tom Vink, Margreet Jonker, Bert A 't Hart, Marina Mané-Damas, Peter C Molenaar, Pilar Martinez-Martinez, Eline van der Esch, Janine Schuurman, Marc H de Baets, Paul W H I Parren
Autoantibodies against ion channels are the cause of numerous neurologic autoimmune disorders. Frequently, such pathogenic autoantibodies have a restricted epitope-specificity. In such cases, competing antibody formats devoid of pathogenic effector functions (blocker antibodies) have the potential to treat disease by displacing autoantibodies from their target. Here, we have used a model of the neuromuscular autoimmune disease myasthenia gravis in rhesus monkeys (Macaca mulatta) to test the therapeutic potential of a new blocker antibody: MG was induced by passive transfer of pathogenic acetylcholine receptor-specific monoclonal antibody IgG1-637...
April 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28425585/eosinophilic-esophagitis-unclear-roles-of-ige-and-eosinophils
#9
C Blanchard, D Simon, A Schoepfer, A Straumann, H-U Simon
Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the oesophagus. Recognized as a distinct entity only two decades ago, the emergence of the disease along with the availability of new technologies has rapidly opened new research avenues and outlined the main features of the pathogenesis of EoE. Yet, each advance in our understanding of the disease has raised new questions about the previous consensus. Currently, new subsets of the disease challenge our diagnostic criteria. For instance, it was believed that EoE did not respond to proton pump inhibitor (PPI) therapy; however, it has now been shown that a substantial proportion of EoE patients indeed respond to PPIs...
May 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28423973/neurological-involvement-of-igg4-related-disease-description-of-a-case-and-review-of-the-literature
#10
Marco Varrassi, Camilla Gianneramo, Francesco Arrigoni, Paolo Cerrone, Patrizia Sucapane, Carmine Marini, Alessandra Splendiani
IgG4-related disease is a recently discovered pathological entity, histologically characterised by fibrosis and IgG4-positive plasma cell infiltration. This condition may virtually involve every site of the organism, with a various range of clinical presentations. The most commonly affected organ is the pancreatic gland, but it can also involve the biliary tract, salivary and lacrimal glands, kidneys, orbital tissues, lymph nodes, lungs and many others. More recently, IgG4-related disease has been demonstrated to involve, in rare cases, also the central nervous system, with a pattern mainly characterised by hypertrophic pachymeningitis...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28422840/case-report-a-female-case-of-isolated-igg4-related-sclerosing-cholangitis-mimicking-cholangiocarcinoma
#11
Jianchun Xiao, Guanqiao Li, Gang Yang, Congwei Jia, Binglu Li
RATIONALE: IgG4-related disease is a newly recognized fibroinflammatory disorder, characterized by tumefactive lesions, storiform fibrosis and IgG4-positive plasma cells infiltration. IgG4-related sclerosing cholangitis (IgG4-SC) is the most common extrapancreatic manifestation of IgG4-related disease, but it is frequently associated with autoimmune pancreatitis(AIP). Only few case was reported to be diagnosed with IgG4-SC in the absence of AIP, with a striking male preponderance. Here we report a female case of isolated IgG4 related sclerosing cholangitis mimicking cholangiocarcinoma...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28421369/immunophenotypic-profiles-for-distinguishing-orbital-mucosa-associated-lymphoid-tissue-lymphoma-from-benign-lymphoproliferative-tumors
#12
Shunichiro Ueda, Yoshihiko Usui, Takeshi Nagai, Daniel Diaz-Aguilar, Toshitaka Nagao, Hiroshi Goto
PURPOSE: To evaluate novel immunophenotypic profiles of patients with orbital lymphoproliferative tumors. METHODS: From 2006 to 2014, surgical biopsies from consecutive patients with newly diagnosed orbital mucosa-associated lymphoid tissue (MALT) lymphoma and IgG4-related ophthalmic disease (IgG4-ROD) were collected from Tokyo Medical University Hospital. A total of 44 tumors from 44 patients were analyzed, including 21 with orbital MALT lymphoma (11 men and 10 women, mean age 67...
April 18, 2017: Japanese Journal of Ophthalmology
https://www.readbyqxmd.com/read/28420843/rosai-dorfman-disease-of-the-lung-overlapping-with-igg4-related-disease-the-difficulty-in-its-differential-diagnosis
#13
Mizue Hasegawa, Fumikazu Sakai, Asako Okabayashi, Hideki Katsura, Toshiko Kamata, Eitetsu Koh, Yasuo Sekine, Tamiko Takemura, Yukio Nakatani, Kenzo Hiroshima
We herein report a case of Rosai-Dorfman disease (RDD) overlapping with IgG4-related disease (IgG4-RD), which presented as diffuse interstitial lung disease with a perilymphatic pattern, followed by submandibular gland and eyelid swelling. The pathological findings of the submandibular gland biopsy specimen were indicative of IgG4-RD alone. We diagnosed the patient with RDD with overlapping IgG4-RD. However, the optimal method for differentiating between these two entities is still controversial. It is important that clinicians are aware that RDD should be included in the differential diagnoses of diffuse interstitial lung disease with a perilymphatic pattern and that RDD can overlap with IgG4-RD...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28413769/update-on-biomarkers-to-monitor-clinical-efficacy-response-during-and-post-treatment-in-allergen-immunotherapy
#14
REVIEW
Lubna Kouser, Jasper Kappen, Ross P Walton, Mohamed H Shamji
Allergen immunotherapy (AIT) is an immune modulating treatment for allergic diseases. Although highly effective, some patients do not respond to the treatment. To date there are no surrogate biomarkers that are predictive of the clinical response to AIT. More and more is known about the underlying immunological mechanism involved in AIT. Through modulation of both innate and adaptive immune responses, involving reduced ILC2 and enhanced Treg and Breg induction and functionality, along with induction of IgG4 antibody production which have the capacity to inhibit both allergen-induced basophil responsiveness and CD23-mediated IgE-facilitated allergen presentation, the result is an immune skewing towards a more balanced Type I response...
2017: Current Treatment Options in Allergy
https://www.readbyqxmd.com/read/28413094/-pachymeningitis
#15
O Fain, A Mekinian
Pachymeningitis is a fibrosing and inflammatory process, which involves the dura mater. Some pachymeningitis are cranial and induce headaches and cranial nerve palsies. Others are spinal and responsible for nerve roots or spinal cord compression. MRI shows contrast enhancement thickening of the dura mater. Etiologies are infectious (syphilis, tuberculosis, etc.) or inflammatory (sarcoidosis, granulomatosis with polyangiitis, IgG4-related disease, idiopathic). Corticosteroids are the main treatment. The use of immunosuppressive drugs or rituximab is yet to be determined and probably adapted to each etiology...
April 13, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28412148/no-evidence-to-support-a-role-for-helicobacter-pylori-infection-and-plasminogen-binding-protein-in-autoimmune-pancreatitis-and-igg4-related-disease-in-a-uk-cohort
#16
Emma L Culver, Wouter L Smit, Caroline Evans, Ross Sadler, Tamsin Cargill, Mateusz Makuch, Lai-Mun Wang, Berne Ferry, Paul Klenerman, Eleanor Barnes
BACKGROUND AND OBJECTIVES: Helicobacter pylori (H.pylori) plasminogen binding protein (PBP) has been proposed as an antigen triggering autoimmune pancreatitis (AIP), the pancreatic manifestation of IgG4-related disease (IgG4-RD). We investigated exposure to H. pylori infection, cytokine response and immunological memory to H. pylori PBP in a prospective IgG4-RD cohort in the UK. METHODS: Clinical and endoscopic evidence of peptic ulceration, serological H. pylori exposure and serum IgG4 levels were obtained in 55 IgG4-RD patients and 52 disease controls (DC) with autoimmune or inflammatory conditions with an elevated serum IgG4...
April 5, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/28401875/-tumors-of-unknown-origin-case-report
#17
Arni Jon Geirsson, Sigridur Thordis Valtysdottir, Andres Sigvaldason, Margret Sigurdardottir
IgG4 related disease is a recently recognized chronic fibrotic, inflammatory condition, caused by infiltrating IgG4 positive plasma cells that can cause tumor like disease in almost any organ in the body. Typical histopathology is lymphoplasmocytic infiltration of IgG4 positive cells, storiform fibrosis and obliterative phlebitis. Glucocorticoids alone or in combination with B-cell depletion with rituximab causes often good, lasting response. We present here a lady with recurrent lung infiltration that simulated pneumonia and later tumor of the lung...
April 2017: Læknablađiđ
https://www.readbyqxmd.com/read/28394784/igg4-related-sclerosing-disease-involving-middle-ear
#18
Ping Lu, Yan Sha, Feng Wang, Shuyi Wang
No abstract text is available yet for this article.
April 7, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28393980/-igg4-associated-nephritis-and-recurrent-hemoptysis-case-report
#19
Daniel Erlij, Ángela Rivera, Juan Carlos Maya, Carolina Cuellar, Gonzalo Correa, Susana Michalland, Gonzalo P Méndez
IgG4 disease is a multi-systemic condition involving pancreas, salivary glands and lymph nodes. Less frequently, it causes interstitial nephritis and involves the lungs. We report a 58 years old male with a four years history of hemoptysis and renal dysfunction characterized by hematuria and proteinuria, responsive to steroidal therapy. The renal biopsy established the diagnosis of IgG4 associated interstitial nephritis. Lung involvement was considered secondary to the same systemic disease.
January 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28392497/jugular-foramen-collision-tumor-schwannoma-and-plasma-cell-pseudotumor-a-probable-igg4-related-disease-a-case-report
#20
Mudathir S Bakhit, Masazumi Fujii, Shinya Jinguji, Taku Sato, Jun Sakuma, Kiyoshi Saito
Lower cranial nerve sheath tumors are relatively rare, and cases of schwannoma collision tumors have rarely been reported, with most of the reported cases describing schwannoma and meningioma collision tumors. We report a very rare case of a cerebellopontine angle collision tumor of the 9(th) cranial nerve schwannoma with an IgG4 plasma cell pseudotumor. IgG4 plasma cell pseudotumors comprise a group of diseases called IgG4-related diseases (IgG4-RDs). These diseases usually affect organs such as the pancreas and salivary gland...
April 6, 2017: World Neurosurgery
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