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"IgG4-related disease"

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https://www.readbyqxmd.com/read/28806261/unexpected-fibrosing-mediastinitis-shown-on-fdg-pet-ct-in-a-patient-with-igg4-related-disease
#1
Ying Kan, Leilei Yuan, Wei Wang, Jigang Yang
A 66-year-old man presented to our hospital because of abdominal pain for 5 days. A contrast abdominal CT raised the possibility of pancreatic carcinoma. FDG PET/CT showed increased FDG accumulation not only in the pancreas and the retroperitoneum, but also in the posterior mediastinum, which was not typical of pancreatic carcinoma. The patient was subsequently diagnosed having immunoglobulin G4-related disease following the histopathologic examination.
August 12, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28806184/noninfectious-aortitis-what-the-cardiologist-needs-to-know
#2
Ingolf Töpel
PURPOSE OF REVIEW: To sum up a group of noninfectious inflammatory diseases of the aorta and its branches relevant to a cardiologist's daily routine. To describe pathogenetic and clinical advances as well as modern diagnostic tools. To overview most recent treatment options and patient-tailored therapies. To provide an insight in future directions of research. RECENT FINDINGS: Pathophysiology of large vessel vasculitides (LVV) are still poorly defined. At least a certain number of patients with idiopathic periaortitis seem to part of the group of IgG4-related diseases which has implications for therapy...
August 12, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28803167/igg4-related-disease-a-new-etiology-underlying-diffuse-intracranial-dilating-vasculopathy
#3
Evan S Marlin, Davis Dornbos, Daniel S Ikeda, Norman L Lehman, Ciarán J Powers
BACKGROUND: Diffuse intracranial aneurysmal vasculopathy is a rare condition, previously described in patients with human immunodeficiency virus (HIV) infection. IgG4 related disease (IgG4-RD) is a recognized inflammatory disease of systemic organs, leading to fibrosis of connective tissues. It has also been linked to inflammatory dilating aortic aneurysms, coronary vascular disease, hypophysitis, orbital pseudotumor and pachymeningitis. It has not yet been described as a cause of diffuse intracranial dilating vasculopathy...
August 9, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28800014/large-tumefactive-igg4-related-disease-histologic-cytologic-and-immunohistochemical-features-of-a-very-unusual-case
#4
Israh Akhtar, Veena Shenoy, Majid Khan, Ali G Saad
Immunoglobulin G4-related disease (IgG4-RD) is a regional or systemic multiorgan lymphoplasmacytic inflammatory disease of unknown etiology. It has been described in numerous organs and anatomic locations. Review of the literature shows that when the disease involves the retroperitoneum it causes retroperitoneal fibrosis. Tumefactive IgG4-RD of the retroperitoneum has not been previously reported. In this report, we describe the first case of a large retroperitoneal tumefactive IgG4-RD along with its histologic, cytologic, and immunohistochemical characteristics...
August 9, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28782655/on-the-role-of-igg4-in-inflammatory-conditions-lessons-for-igg4-related-disease
#5
REVIEW
David C Trampert, Lowiek M Hubers, Stan F J van de Graaf, Ulrich Beuers
The pathophysiology of immunoglobulin G4-related disease (IgG4-RD) and its most common manifestations, IgG4-associated (sclerosing) cholangitis and autoimmune pancreatitis, remains largely unknown, but IgG4 is presumably involved. IgG4 is a promiscuous antibody, which could be directly pathogenic, fulfill a protective role, or could just be a fortuitous marker of an aberrant inflammatory response. IgG4 antibodies possess exclusive structural and functional characteristics suggesting anti-inflammatory and tolerance-inducing effects...
August 3, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28780079/antineutrophil-cytoplasmic-antibody-associated-vasculitides-and-igg4-related-disease-a-new-overlap-syndrome
#6
REVIEW
François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
OBJECTIVE: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. METHODS: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria...
August 2, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28777155/a-splenic-igg4-sclerosing-angiomatoid-nodular-transformation-sant-treated-by-hemisplenectomy-a-radiologic-histochemical-and-immunohistochemical-study
#7
Raffaele Gaeta, Francescamaria Donati, Emanuele F Kauffmann, Daniela Campani
INTRODUCTION: Sclerosing angiomatoid nodular transformation (SANT) is a rare benign lesion of unknown origin for which total splenectomy is the standard treatment. CASE PRESENTATION: A 54-year-old man with a history of recurrent pancreatitis, bicuspid aortic valve, and aortic dissection underwent abdominal ultrasound, Computed tomography and magnetic resonance imaging, which revealed a 6-cm hypoechoic splenic mass diagnosed as cavernous hemangioma. Owing to his relevant past history, he was considered eligible for emisplenectomy and not for total excision, which is associated with long-term risks, especially infections...
August 2, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28768471/unsuspected-human-immunodeficiency-virus-infection-presenting-as-immunoglobulin-g4-related-lymphadenopathy-a-case-report
#8
Hsing-Tse Yu, Chen-Hsiang Lee, Shun-Chen Huang, Shan-Fu Yu
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition characterized by infiltration of the involved organs by IgG4-bearing plasma cells. The prevalence of autoimmune diseases, associated with or occurring in patients with human immunodeficiency virus (HIV) infection, has been increasing. We describe a 58-year-old man with an undiagnosed HIV infection, which presented as chronic cervical lymphadenopathy with an elevated serum IgG4 and a very high IgE. Histologically, lymph nodes showed expanded sinusoids and burnt-out germinal centers with increased plasmacytic infiltration and collagen fiber deposition...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28768224/igg4-related-hypertrophic-pachymeningitis-of-the-spine-with-mpo-anca-seropositivity
#9
Mary Maher, George Zanazzi, Phyllis Faust, Katherine Nickerson, Tony T Wong
IgG4-related disease is an immune-mediated fibro-inflammatory condition that affects multiple organ systems. When the pachymeninges are involved, it is termed IgG4-related hypertrophic pachymeningitis (IgG4-RHP). We present a 79-year-old woman with back pain found to have IgG4-RHP localized exclusively in the spine who was also seropositive for myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA). This case is the first known report of IgG4-RHP in the spine with MPO-ANCA and adds to emerging evidence for potential disease overlap in the pathogenesis of hypertrophic pachymenigitis (HP)...
July 27, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28765476/annexin-a11-is-targeted-by-igg4-and-igg1-autoantibodies-in-igg4-related-disease
#10
Lowiek M Hubers, Harmjan Vos, Alex R Schuurman, Robin Erken, Ronald P Oude Elferink, Boudewijn Burgering, Stan F J van de Graaf, Ulrich Beuers
OBJECTIVE: Immunoglobulin G4-related disease (IgG4-RD) is a multiorgan immune-mediated disease that predominantly affects the biliary tract (IgG4-associated cholangitis, IAC) and pancreas (autoimmune pancreatitis, AIP). We recently identified highly expanded IgG4+ B-cell receptor clones in blood and affected tissues of patients with IAC/AIP suggestive of specific (auto)antigenic stimuli involved in initiating and/or maintaining the inflammatory response. This study aimed to identify (auto)antigen(s) that are responsible for the clonal expansion of IgG4+ B cells in IgG4-RD...
August 1, 2017: Gut
https://www.readbyqxmd.com/read/28749851/immunoglobulin-g4-igg4-positive-ocular-adnexal-mucosa-associated-lymphoid-tissue-lymphoma-and-idiopathic-orbital-inflammation
#11
Eun Jung Sohn, Hee Bae Ahn, Mee Sook Roh, Woo Jun Jung, Won Yeol Ryu, Yoon Hyung Kwon
PURPOSE: To report clinical and pathological characteristics of idiopathic orbital inflammation and ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma with immunoglobulin G4 (IgG4)-positive plasma cells. METHODS: A retrospective histopathological review and clinical case series. A total of 51 biopsy samples from January 2005 to December 2015 were used in this study, including 21 cases of biopsy-confirmed idiopathic orbital inflammation and 30 cases of biopsy-confirmed ocular adnexal MALT lymphoma...
July 26, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28747608/igg4-related-disease-mechanistic-insights-from-both-clinical-and-immunologic-understanding-of-this-condition
#12
Takashi Maehara
  IgG4-related disease (IgG4-RD) is a chronic inflammatory disease characterized by tumescent lesions with characteristic storiform fibrosis, obliterative phlebitis and a marked lymphoplasmacytic infiltrate that includes a large number of IgG4 positive plasma cells. It's widely accepted that rituximab-mediated B cell depletion therapy is effective for this disease. Important mechanistic insights correlated with the pathogenesis of IgG4-RD have been gradually disclosed from studies of patients treated by B cell depletion...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28744088/igg4-related-disease-simulating-paraneoplastic-syndrome-role-of-18-fdg-pet-ct-imaging
#13
Madhuri S Mahajan, Sudeshna Maitra, Natasha Singh, Melvika Pereira
Immunoglobulin G4 (IgG4)-related systemic disease (IgG4-RSD) is a new systemic entity associated with autoimmune pancreatitis (AIP). Other organ involvements take the form of sclerosing cholangitis, sclerosing cholecystitis, sclerosing sialadenitis, retroperitoneal fibrosis, and interstitial nephritis. Recently, lung diseases related to IgG4 have been described to occur with or without other organ involvement. These diseases include interstitial lung disease (ILD), pulmonary inflammatory pseudotumor, and lymphomatoid granulomatosis...
April 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/28736430/a-case-of-type-1-autoimmune-pancreatitis-aip-a-form-of-igg4-related-disease-igg4-rd
#14
Mounira El Euch, Souha Hddad, Madiha Mahfoudhi, Hela Maktouf, Fethi Ben Hamida, Fatima Jaziri, Khaoula Ben Abdelghani, Sami Turki, Taïeb Ben Abdallah
BACKGROUND Type 1 autoimmune pancreatitis (AIP), also known as lymphoplasmacytic sclerosing pancreatitis (LPSP), is a rare cause of chronic pancreatitis, characterized by a fibro-inflammatory process. However, patients with AIP may have a good response to corticosteroid therapy. We describe a Tunisian patient with AIP that was confirmed to be an IgG4-related disease (IgG4-RD). CASE REPORT We describe a case of a 70-year-old man who was admitted to hospital for obstructive jaundice and abdominal pain. Serum liver function tests were abnormal and upper abdominal computed tomography (CT) imaging showed diffuse pancreatic swelling and strictures of the main pancreatic duct without any focal lesion...
July 24, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28735232/immunoglobulin-g4-related-mastitis-a-case-report
#15
Ee Syn Tan, Brendon Friesen, Seow Foong Loh, Jane Fox
INTRODUCTION: IgG4-related mastitis (IgG4-RM) is exceedingly rare with only ten cases reported in the literature to date. Organs that are affected with IgG4-related disease (IgG4-RD) all share the same histopathological hallmarks consisting of dense lymphocytic infiltration, storiform fibrosis and obliterative phlebitis. PRESENTATION OF CASE: This case report highlights a case of IgG4-RM found incidentally in a 52-year-old woman during a routine breast screen and it explores the current literature about IgG4-RM and IgG4-RD...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28733656/efficacy-of-cyclophosphamide-treatment-for-immunoglobulin-g4-related-disease-with-addition-of-glucocorticoids
#16
Fei Yunyun, Chen Yu, Zhang Panpan, Chen Hua, Wu Di, Zhao Lidan, Peng Linyi, Wang Li, Wu Qingjun, Zhang Xuan, Zhao Yan, Zeng Xiaofeng, Zhang Fengchun, Zhang Wen
Aim to evaluate the efficacy and safety of glucocorticoid monotherapy vs combination therapy of cyclophosphamide (CYC) for IgG4 related disease (IgG4-RD). 102 newly diagnosed IgG4-RD patients were enrolled and assigned to 2 groups: Group I was prednisone monotherapy (0.5-1.0 mg/kg.d, tapered gradually) and Group II was glucocorticoid and CYC (50-100 mg per day). Patients were assessed at different periods. Primary end point was relapse rate; secondary end points included response, remission rate and adverse effects...
July 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28720475/a-case-of-igg4-related-disease-presenting-with-multiple-gastric-submucosal-tumors
#17
Shoichiro Mine, Eisuke Ozawa, Ken Ohnita, Kuniko Abe, Kazuhiko Nakao
No abstract text is available yet for this article.
July 15, 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28717084/pathologically-proven-spontaneous-remission-of-igg4-related-retroperitoneal-fibrosis
#18
Hideaki Yamakawa, Akimasa Sekine, Yumie Yamanaka, Shinko Sadoyama, Tomohisa Baba, Eri Hagiwara, Koji Okudela, Takashi Ogura
Some forms of idiopathic retroperitoneal fibrosis (RF) have recently been considered to be a part of the spectrum of immunoglobulin G4 (IgG4)-related disease. This case report is the first description of a spontaneous remission in a patient with pathologically proven IgG4-related RF. Although the pathogenesis and long-term disease behavior of IgG4-related RF remains unknown, we believe that an initial assessment consisting of only careful monitoring might be one important strategy, especially in asymptomatic IgG4-related RF patients without nephropathy, while carefully monitoring these patients for the risk of recurrence...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28716871/igg4-related-disease-long-term-natural-history-and-management-of-a-relapsing-multisystem-disease-entity
#19
Sreelakshmi Kotha, Giovanni Tritto, Terry Wong, Philip Berry
Although awareness of IgG4-related disease has grown over the past decade, with earlier diagnosis and treatment, understanding of its natural history over the long term and the optimal management remains unclear. We report the case of a 48-year-old man who presented with a pancreatic pseudotumour causing bile duct obstruction with coexisting autoimmune hepatitis and multisystem involvement. His symptoms settled on steroids and maintenance with azathioprine was commenced, however periodic relapses occurred involving multiple organs...
July 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28710703/igg4-related-disease-involving-polyserous-effusions-with-elevated-serum-interleukin-6-levels-a-case-report-and-literature-review
#20
Xiang Tong, Min Bai, Weiya Wang, Qingbing Han, Panwen Tian, Hong Fan
Immunoglobulin G4-related disease (IgG4-RD) is a recently described immune-mediated fibroinflammatory disease with a characteristic histopathologic appearance that can affect various organs. We report a 43-year-old Chinese female patient with IgG4-RD involving polyserous effusions with reports of worsening exertional dyspnea for 3 months. Laboratory blood tests revealed that serum interleukin (IL)-6, carbohydrate antigens (CA-199 and CA-125), and alpha-fetoprotein levels were significantly increased, but serum IgG4 levels were normal...
August 2017: Immunologic Research
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