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IgG4 disease

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https://www.readbyqxmd.com/read/28811201/the-unmet-need-in-rheumatology-reports-from-the-targeted-therapies-meeting-2017
#1
Kevin L Winthrop, Vibeke Strand, Désirée van der Heijde, Philip Mease, Mary Crow, Michael Weinblatt, Joan Bathon, Gerd R Burmester, Maxime Dougados, Johnathan Kay, Xavier Mariette, Ronald Van Vollenhoven, Joachim Sieper, Fritz Melchers, Ferdinand C Breedfeld, Joachim Kalden, Josef S Smolen, Daniel E Furst
The 19th annual international Targeted Therapies meeting brought together over 100 leading basic scientists and clinical researchers from around the world in the field of immunology, molecular biology and rheumatology and other specialties. During the meeting, breakout sessions were held consisting of 5 disease-specific groups with 20-40 experts assigned to each group based on clinical or scientific expertise. Specific groups included: rheumatoid arthritis, psoriatic arthritis, axial spondyloarthritis, systemic lupus erythematous, connective tissue diseases (e...
August 12, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28807714/systemic-igg4-related-disease-in-an-asymptomatic-patient
#2
Fernando A Camporro, Exequiel Bulacio, Ignacio Gutierrez Magaldi
No abstract text is available yet for this article.
August 11, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28806261/unexpected-fibrosing-mediastinitis-shown-on-fdg-pet-ct-in-a-patient-with-igg4-related-disease
#3
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
#4
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/28805160/clinical-implications-of-immunoglobulin-g4-to-graves-ophthalmopathy
#5
Sung Hoon Yu, Jun Goo Kang, Chul Sik Kim, Sung-Hee Ihm, Moon Gi Choi, Hyung Joon Yoo, Seong Jin Lee
BACKGROUND: This study aimed to explore relation of immunoglobulin G4 (IgG4) to clinical and laboratory characteristics of patients newly diagnosed with Graves' disease (GD) without or with Graves' ophthalmopathy (GO), and to analyze association of IgG4 with development and grade of GO in GD patients. METHODS: Sixty-four GD patients and 64 sex- and age-matched euthyroid subjects were enrolled. Serum levels of thyroid hormones, thyroid autoantibodies, immunoglobulin G (IgG) and IgG4 were measured, and ophthalmological and ultrasonographical evaluation was performed...
August 12, 2017: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/28803167/igg4-related-disease-a-new-etiology-underlying-diffuse-intracranial-dilating-vasculopathy
#6
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
#7
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/28799078/intrachiasmatic-abscess-caused-by-igg4-related-hypophysitis
#8
Georgios F Hadjigeorgiou, Eva Løbner Lund, Lars Poulsgaard, Ulla Feldt-Rasmussen, Åse Krogh Rasmussen, Marianne Wegener, Kåre Fugleholm
INTRODUCTION: Autoimmune hypophysitis is a rare disease of the pituitary, which may affect vision by inflammation and compression of the optic chiasm. However, intrachiasmatic abscess formation has not been previously reported. METHODS: In this study, we report a case of a 29-year-old female with bitemporal hemianopia due to a cystic intrasellar tumor. The patient underwent surgical decompression of the lesion, which was found to be an intrachiasmatic abscess. RESULTS: The histologic findings were consistent with IgG4 hypophysitis...
August 10, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28795528/limit-of-detection-and-threshold-for-positivity-of-the-centers-for-disease-control-and-prevention-assay-for-factor-viii-inhibitors
#9
Connie H Miller, Brian Boylan, Amy D Shapiro, Steven R Lentz, Brian M Wicklund
BACKGROUND: The Bethesda assay (BA) for measurement of factor VIII (FVIII) inhibitors called for quantitation of positive inhibitors using dilutions producing 25-75% residual activity (RA), corresponding to 0.4-2.0 Bethesda units, recommending use of "more sensitive methods" for samples with RA closer to 100%. The Nijmegen modification (NBA) changed the reagents used but not these calculations. Some specimens negative by NBA have been shown to have FVIII antibodies detectable by sensitive immunologic methods...
August 10, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28782655/on-the-role-of-igg4-in-inflammatory-conditions-lessons-for-igg4-related-disease
#10
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
#11
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
#12
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/28774276/successful-treatment-of-highly-advanced-immunoglobulin-g4-related-kidney-disease-presenting-renal-mass-like-regions-with-end-stage-kidney-failure-a-case-study
#13
Hiroyuki Ono, Taichi Murakami, Akira Mima, Eriko Shibata, Masanori Tamaki, Sakiya Yoshimoto, Sayo Ueda, Fumi Kishi, Seiji Kishi, Takashi Kawanaka, Motokazu Matsuura, Kojiro Nagai, Hideharu Abe, Masashi Harada, Toshio Doi
BACKGROUND: Immunoglobulin G4-related kidney disease characterized by immunoglobulin G4-positive plasma cell-rich tubulointerstitial nephritis has distinctive serological and radiological findings. Renal prognosis is good because of a good response to glucocorticoids. Here we report a case of successful treatment of highly advanced immunoglobulin G4-related kidney disease presenting renal mass-like regions with end-stage kidney failure. CASE PRESENTATION: A 59-year-old Japanese man was referred to our hospital because of uremia with a creatinine level of 12...
August 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28768471/unsuspected-human-immunodeficiency-virus-infection-presenting-as-immunoglobulin-g4-related-lymphadenopathy-a-case-report
#14
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
#15
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
#16
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
#17
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/28749002/-natalizumab-for-the-treatment-of-crohn%C3%A2-s-disease-report-of-three-cases
#18
Daniela Fluxá, Patricio Ibáñez, Lilian Flores, Carolina Figueroa, Jaime Lubascher, Udo Kronberg, Daniela Simian, Gonzalo Pizarro, Paola Toche, Rodrigo Quera
Anti-tumor necrosis factor-α (TNF) agents have dramatically changed the management of Crohn’s Disease (CD). However, a significant number of these patients do not respond at all or cease to respond to antibodies against TNF. In this clinical situation, the options include intensification of anti-TNF therapy by either increasing the dose or by shortening the administration interval, the use of a second anti-TNF or medications with a different mechanism of action. Among the later, Natalizumab, a humanized IgG4 monoclonal antibody against α4β1 and α4β7 integrins, is safe and effective in inducing and maintaining remission in active CD patient’s refractory to anti-TNF...
April 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28747608/igg4-related-disease-mechanistic-insights-from-both-clinical-and-immunologic-understanding-of-this-condition
#19
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/28747266/igg4-complement-and-the-mechanisms-of-blister-formation-in-pemphigus-and-bullous-pemphigoid
#20
REVIEW
Teruki Dainichi, Zachary Chow, Kenji Kabashima
Autoimmune bullous diseases are at the forefront of the research field on autoimmune diseases. Pemphigus and pemphigoid were historical entities in the world of descriptive dermatology for a long time. Recently, however, dermatologists and skin biologists have elegantly explained the novel pathomechanism of pemphigus and pemphigoid diseases. IgG4 is the major subclass of autoantibodies in autoimmune bullous diseases and is known to have little activity to activate complement. It is quite acceptable for pemphigus, because acantholysis in pemphigus has been demonstrated to be complement-independent...
July 18, 2017: Journal of Dermatological Science
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