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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/28420843/rosai-dorfman-disease-of-the-lung-overlapping-with-igg4-related-disease-the-difficulty-in-its-differential-diagnosis
#1
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/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
#2
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/28375417/igg4-aortitis-a-case-report
#3
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
#4
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/28365915/autoimmune-pancreatitis
#5
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/28351925/serum-igg2-and-tissue-igg2-plasma-cell-elevation-in-orbital-igg4-related-disease-igg4-rd-potential-use-in-igg4-rd-assessment
#6
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
#7
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/28321964/combination-therapy-of-leflunomide-and-glucocorticoids-for-the-maintenance-of-remission-in-patients-with-igg4-related-disease-a-retrospective-study-and-literature-review
#8
Yiwen Wang, Kunpeng Li, Dai Gao, Gui Luo, Yurong Zhao, Xiuru Wang, Jie Zhang, Jingyu Jin, Zheng Zhao, Chunhua Yang, Jian Zhu, Jianglin Zhang, Feng Huang
BACKGROUND: Although glucocorticoids are effective in IgG4-related disease (IgG4-RD), patients may relapse during or after glucocorticoid tapering. Immunosuppressive agents including leflunomide (LEF) are regarded as steroid-sparing agents in other autoimmune disorders and need to be discussed in the management of IgG4-RD. AIM: To identify the efficacy and safety of combination therapy of LEF and glucocorticoids in IgG4-RD. METHODS: We retrospectively summarized data of patients diagnosed with IgG4-RD between November 2012 and November 2015...
March 21, 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28319486/igg4-related-disease
#9
Emanuele Bozzalla Cassione, John H Stone
PURPOSE OF REVIEW: Remarkable insights have been gleaned recently with regard to the pathophysiology of IgG4-related disease (IgG4-RD). These findings have direct implications for the development of targeted strategies for the treatment of this condition. RECENT FINDINGS: Oligoclonal expansions of cells of both the B and T lymphocyte lineages are present in the blood of patients with IgG4-RD. Oligoclonal expansions of plasmablasts are a good biomarker for disease activity...
May 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28304239/immunoglobulin-g4-related-epidural-inflammatory-pseudotumor-presenting-with-pulmonary-complications-and-spinal-cord-compression-case-report
#10
Kavelin Rumalla, Kyle A Smith, Paul M Arnold
Immunoglobulin G4-related disease (IgG4-RD) is a recently defined condition characterized by inflammatory tumefactive lesions in various organ systems. IgG4-RD is a clinical and radiological diagnosis of exclusion and requires the presence of specific histopathological criteria for diagnosis. A 50-year-old man presented to an outside hospital with a 3-month history of progressively worsening back pain and symptoms of pleurisy, nasal crusting, and hematochezia. Radiological workup revealed an epidural-paraspinal mass with displacement of the spinal cord, destruction of the T5-6 vertebrae, and extension into the right lung...
March 17, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28297756/-igg4-immunohistochemistry-in-riedle-thyroiditis
#11
S Wang, Y F Luo, J L Cao, H Zhang, X H Shi, Z Y Liang, R E Feng
Objective: To observe the histopathological changes and immunohistochemical expression of IgG4 in Riedle thyroiditis (RT) and to study the relationship between RT and IgG4-related diseases (IgG4-RD). Methods: A total of 5 RT patients were collected from the Department of Pathology, Peking Union Medical College Hospital during April 2012 to August 2014. The clinical and immunohistochemical features were analyzed in the 5 patients. Histopathologic analysis was performed on hematoxylin and eosin-stained sections...
March 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28284385/igg4-related-tubulointerstitial-nephritis
#12
REVIEW
Pingchuan Zhang, Lynn D Cornell
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fibroinflammatory disorder that can involve nearly any organ. The disorder has increasingly become known as a distinct clinical entity during the last decade. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-RD in the kidney. Many patients with IgG4-TIN are diagnosed after IgG4-RD has been recognized in other organ systems, but the kidney may also be the first or only site involved. The presenting clinical features of IgG4-TIN are most commonly kidney insufficiency, kidney mass lesion(s), or both...
March 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/28284351/is-musk-myasthenia-gravis-linked-to-igg4-related-disease
#13
Pooja Raibagkar, Judith A Ferry, John H Stone
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition that affects a wide variety of sites, including the nervous system, where it can involve the meninges or the pituitary gland, and cause perineural mass lesions. A large subset of acetylcholine receptor antibody (Ab)-negative myasthenia gravis (MG) patients has muscle-specific tyrosine kinase (MuSK) Abs, generally of the IgG4 subclass. There has not been any association found between IgG4-RD and MuSK MG yet. We report the first case of MuSK MG associated with lymphadenopathy with histopathology consistent with IgG4-RD...
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28272212/ophthalmic-manifestations-in-igg4-related-disease-clinical-presentation-and-response-to-treatment-in-a-french-case-series
#14
MULTICENTER STUDY
Mikael Ebbo, Matthieu Patient, Aurelie Grados, Matthieu Groh, Julien Desblaches, Eric Hachulla, David Saadoun, Sylvain Audia, Aude Rigolet, Benjamin Terrier, Antoinette Perlat, Constance Guillaud, Frederic Renou, Emmanuelle Bernit, Nathalie Costedoat-Chalumeau, Jean-Robert Harlé, Nicolas Schleinitz
IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series.Patients with IgG4-RD and orbital or orbital adnexa involvement included in the French multicentric IgG4-RD case-registry were identified. Only patients fulfilling "modified" comprehensive diagnostic criteria with pathological documentation were retained for the study...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28256769/immunoglobulin-g4-related-disease-and-its-skin-manifestations
#15
REVIEW
Ryoko Takayama, Takashi Ueno, Hidehisa Saeki
Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory condition characterized by tissue infiltration with IgG4(+) plasma cells and an elevated serum IgG4 level. It usually occurs in middle-aged or older patients, with male predominance, and can involve multiple organs synchronously or metachronously. Patients with IgG4-RD generally respond to steroid therapy. IgG4-related skin lesions are typically erythematous and itchy plaques, subcutaneous nodules, or papules like prurigo nodularis located on the head and neck areas...
March 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28244022/characteristics-of-immunoglobulin-g4-related-aortitis-periaortitis-and-periarteritis-on-fluorodeoxyglucose-positron-emission-tomography-computed-tomography-co-registered-with-contrast-enhanced-computed-tomography
#16
Satoshi Yabusaki, Noriko Oyama-Manabe, Osamu Manabe, Kenji Hirata, Fumi Kato, Noriyuki Miyamoto, Yoshihiro Matsuno, Kohsuke Kudo, Nagara Tamaki, Hiroki Shirato
BACKGROUND: We aimed to assess the positivity, distribution, quantitative degree of vessel inflammation, and clinical characteristics of IgG4-related aortitis/periarteritis and periarteritis (IgG4-aortitis), and to examine the difference in these characteristics between cases with and without IgG4-aortitis, using fluorodeoxyglucose positron-emission tomography/computed tomography (FDG-PET/CT) co-registered with contrast-enhanced CT (CECT). We retrospectively evaluated 37 patients with IgG4-related disease (IgG4-RD) who underwent both FDG-PET/CT and CECT...
December 2017: EJNMMI Research
https://www.readbyqxmd.com/read/28243891/igg4-related-disease-presenting-as-panuveitis-without-scleral-involvement
#17
REVIEW
Kinda Najem, Larissa Derzko-Dzulynsky, Edward A Margolin
BACKGROUND: The following case emphasizes the importance of including IgG4-related disease (RD) in the differential diagnosis of intraocular inflammation and multiple cranial nerve palsies. RESULTS: A 33-year-old man, with a history of idiopathic bilateral panuveitis, presented with a new right pupillary-sparing partial third nerve palsy, which spontaneously resolved in 2 weeks, but was followed 1 month later, by a right sixth nerve palsy, which also resolved within a few weeks...
December 2017: Journal of Ophthalmic Inflammation and Infection
https://www.readbyqxmd.com/read/28238541/igg4-related-sclerosing-cholangitis-overlapping-with-autoimmune-hepatitis-report-of-a-case
#18
Hongyan Li, Li Sun, David R Brigstock, Lina Qi, Runping Gao
BACKGROUND: IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-related disease (IgG4-RD) but the presence of IgG4-SC in the porta hepatis is difficult to differentiate from hilar cholangiocarcinoma (HCCA). IgG4-related autoimmune hepatitis (IgG4-related AIH) is extremely rare and it is not fully clear whether IgG4-related AIH is a hepatic manifestation of IgG4-RD or a subtype of AIH. CASE PRESENTATION: We present a rare case of a 52-year-old male who was admitted with obstructive jaundice and itchy skin...
February 10, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28223204/increases-in-ige-eosinophils-and-mast-cells-can-be-used-in-diagnosis-and-to-predict-relapse-of-igg4-related-disease
#19
Emma L Culver, Ross Sadler, Adrian C Bateman, Mateusz Makuch, Tamsin Cargill, Berne Ferry, Rob Aalberse, Eleanor Barnes, Theo Rispens
BACKGROUND & AIMS: Immunoglobulin G4-related disease (IgG4-RD) is characterized by increased serum levels of IgG4 and infiltration of biliary, and pancreatic, and other tissues by IgG4-positive plasma cells. We assessed the prevalence of allergy and/or atopy, serum and tissue IgE antibodies, and blood and tissue eosinophils in patients with IgG4-RD. We investigated the association between serum IgE and diagnosis and relapse of this disease. METHODS: We performed a prospective study of 48 patients with IgG4-RD, 42 patients with an increased serum level of IgG4 with other inflammatory and autoimmune conditions (disease controls), and 51 healthy individuals (healthy controls) recruited from Oxford, United Kingdom from March 2010 through March 2014, and followed for a median of 41 months (range 3-73 months)...
February 18, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28207938/comparison-of-clinical-and-pathological-features-of-lung-lesions-of-systemic-igg4-related-disease-and-idiopathic-multicentric-castleman-s-disease
#20
Yasuhiro Terasaki, Soichiro Ikushima, Shoko Matsui, Akira Hebisawa, Yasunori Ichimura, Shinyu Izumi, Masuo Ujita, Machiko Arita, Keisuke Tomii, Yuko Komase, Isoko Owan, Tetsuji Kawamura, Yasuo Matsuzawa, Miho Murakami, Hiroshi Ishimoto, Hiroshi Kimura, Masashi Bando, Norihiro Nishimoto, Yoshinori Kawabata, Yuh Fukuda, Takashi Ogura
AIMS: The lung lesion [immunoglobulin (Ig)G4-L] of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion [idiopathic multicentric Castleman's disease (iMCD-L)] of idiopathic multicentric Castleman's disease (iMCD). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data. METHODS AND RESULTS: Nine patients had IgG4-L (high levels of serum IgG4 and of IgG4(+) cells in lung specimens; typical extrapulmonary manifestations)...
February 16, 2017: Histopathology
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