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IgG4 related disease

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https://www.readbyqxmd.com/read/28434701/upregulated-interleukins-il-6-il-10-and-il-13-in-immunoglobulin-g4-related-aortic-aneurysm-patients
#1
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/28423973/neurological-involvement-of-igg4-related-disease-description-of-a-case-and-review-of-the-literature
#2
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
#3
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
#4
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
#5
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/28413094/-pachymeningitis
#6
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
#7
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
#8
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
#9
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/28392497/jugular-foramen-collision-tumor-schwannoma-and-plasma-cell-pseudotumor-a-probable-igg4-related-disease-a-case-report
#10
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
https://www.readbyqxmd.com/read/28375417/igg4-aortitis-a-case-report
#11
Shivali Marketkar, Mark LeGolvan
IgG4 aortitis is one of the entities seen in the spectrum of IgG4-related disease (IgG4-RD). It is characterized by serologic (elevated serum IgG4) and histologic features including a lymphoplasmacytic infiltrate with increased numbers of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Some studies have described a correlation between infections and IgG4 aortitis. We describe a patient with an aneurysm of the infrarenal descending abdominal aorta with features of IgG4-RD, as well as culture evidence of Streptococcus sanguis...
April 3, 2017: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/28374231/neurological-manifestations-of-igg4-related-disease
#12
REVIEW
Bernardo Baptista, Alina Casian, Harsha Gunawardena, David D'Cruz, Claire M Rice
IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised-hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required...
April 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28367391/igg4-related-disease-presenting-as-a-pancreatic-mass-and-bilateral-lacrimal-gland-swelling
#13
Uroosa Ibrahim, Amina Saqib, Nikhil Nalluri, Muhammad R Raza, Mark Goldstein
IgG4-related disease is characterized by multi-system inflammation and possible elevation in serum immunoglobulin-G4 level. Treatment regimens include glucocorticoids, disease-modifying anti-rheumatic agents and recently, rituximab infusions have been reported to be effective in treatment-refractory disease. We present a case of a 64-year-old male presenting with acute abdominal pain and scleral icterus. An abdominal ultrasound demonstrated extensive biliary ductal dilatation. A computed tomography (CT) scan and a magnetic resonance cholangiopancreatography (MRCP) revealed a 4...
February 24, 2017: Curēus
https://www.readbyqxmd.com/read/28367047/incipient-ocular-mucosa-associated-lymphoid-tissue-lymphoma-in-igg4-related-orbital-disease
#14
Ji Won Kim, Sol Ah Han, Byeong Jae Son, Myoung Ju Koh, Jin Sook Yoon
No abstract text is available yet for this article.
April 2017: Korean Journal of Ophthalmology: KJO
https://www.readbyqxmd.com/read/28365915/autoimmune-pancreatitis
#15
REVIEW
Shounak Majumder, Naoki Takahashi, Suresh T Chari
Autoimmune pancreatitis (AIP) is a chronic fibroinflammatory disease of the pancreas that belongs to the spectrum of immunoglobulin G-subclass4-related diseases (IgG4-RD) and typically presents with obstructive jaundice. Idiopathic duct-centric pancreatitis (IDCP) is a closely related but distinct disease that mimics AIP radiologically but manifests clinically most commonly as recurrent acute pancreatitis in young individuals with concurrent inflammatory bowel disease. IgG4 levels are often elevated in AIP and normal in IDCP...
April 1, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28359733/right-ethmoid-eosinophilic-angiocentric-fibrosis-with-orbital-extension
#16
S Gorostis, M Bacha, S Gravier, T Raguin
INTRODUCTION: Eosinophilic angiocentric fibrosis (EAF) is a slowly progressive, benign disease involving the mucosa of the upper airways or, more rarely, the orbit. It belongs to the spectrum of IgG4-related disease. CASE REPORT: The authors report the case of a 61-year-old man who presented with orbital involvement (visual loss, pain, proptosis, and eyelid oedema), headache and nasal obstruction. Imaging revealed a right ethmoido-orbital mass infiltrating the periorbital fat and enveloping the optic nerve...
March 27, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28357112/immunoglobulin-g4-related-disease-a-rare-steroid-responsive-disease
#17
Vorawut Thanthitaweewat, Poonchavist Chantranuwatana, Naricha Chirakalwasan
A 70-year-old man presented with progressive dyspnoea and weight loss. Physical examination revealed only mild pale conjunctiva. The workup showed mild anaemia, mild impaired renal function, and high globulin level. Multiple myeloma was excluded by normal serum protein electrophoresis. The chest radiography and computed tomography (CT) revealed bilateral multifocal patchy infiltration with mediastinal adenopathy. Bronchoscopy was performed. Bronchoalveolar lavage (BAL) fluid examination was negative for infection and malignancy...
May 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28351925/serum-igg2-and-tissue-igg2-plasma-cell-elevation-in-orbital-igg4-related-disease-igg4-rd-potential-use-in-igg4-rd-assessment
#18
Anita S Y Chan, Hardeep Mudhar, Sunny Yu Shen, Stephanie S Lang, Malee Fernando, Maryam Hazly Hilmy, Naomi Jayne Guppy, Ian Rennie, Lisa Dunkley, Issam Al Jajeh
AIMS: To determine the role of serum and tissue IgG2 in orbital biopsies with the histological features of IgG4-related disease (IgG4-RD) in comparison with non-IgG4-related orbital inflammatory disorders (OID), including autoimmune disorders. METHODS: This is an international (Sheffield, UK, and Singapore) collaborative, retrospective case review of 69 patients (38 from Singapore National Eye Centre and 31 from Royal Hallamshire Hospital, Sheffield) with orbital inflammatory biopsies between 2002 and 2016...
March 28, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28348556/t-cell-polarization-toward-th2-tfh2-and-th17-tfh17-in-patients-with-igg4-related-disease
#19
Aurélie Grados, Mikael Ebbo, Christelle Piperoglou, Matthieu Groh, Alexis Regent, Maxime Samson, Benjamin Terrier, Anderson Loundou, Nathalie Morel, Sylvain Audia, François Maurier, Julie Graveleau, Mohamed Hamidou, Amandine Forestier, Sylvain Palat, Emmanuelle Bernit, Bernard Bonotte, Catherine Farnarier, Jean-Robert Harlé, Nathalie Costedoat-Chalumeau, Frédéric Vély, Nicolas Schleinitz
IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder involving virtually every organ with a risk of organ dysfunction. Despite recent studies regarding B cell and T cell compartments, the disease's pathophysiology remains poorly understood. We examined and characterized subsets of circulating lymphocytes in untreated patients with active IgG4-RD. Twenty-eight consecutive patients with biopsy-proven IgG4-RD were included in a prospective, multicentric study. Lymphocytes' subsets were analyzed by flow cytometry, with analysis of TH1/TH2/TH17, TFH cells, and cytokine release by peripheral blood mononuclear cells...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28346259/igg4-related-ophthalmic-disease-presenting-as-choroidal-and-orbital-lesions
#20
William S Gange, Stephen M Holland, Felipe De Alba
PURPOSE: To report a case of IgG4-related ophthalmic disease (IgG4-ROD) which presented as choroidal and orbital lesions. METHODS: Case report. RESULTS: A 64-year-old man presented with left eye photopsias and a history of IgG4-related perirenal fibrosis. Fundoscopic examination showed multiple bilateral yellow choroidal lesions, and optical coherence tomography showed multiple choroidal lesions. Magnetic resonance imaging of the orbits showed an enhancing lesion present circumferential to the optic nerve, but greater medially, abutting the posterior surface of the left globe...
March 24, 2017: Retinal Cases & Brief Reports
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