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IgG4 disease

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https://www.readbyqxmd.com/read/28735232/immunoglobulin-g4-related-mastitis-a-case-report
#1
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...
June 15, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28734747/retroperitoneal-fibrosis-associated-with-immunoglobulin-g4-related-disease-in-the-differential-diagnosis-in-retroperitoneal-tumors-case-report
#2
Gabriela Ruiz Mar, Óscar E Cárdenas Serrano, Jorge Roldan García, A Cañavera-Constantino, Víctor M Menéndez Trejo, Óscar Chapa Azuela
The patient was a 55-year-old woman with a 4-month history of pain in left flank that irradiated to the lumbar region. Computed tomography revealed a cystic tumor in the retroperitoneal region that compresses the ureter and left renal pelvis in contact with pancreatic body and tail. Surgery was performed and the definitive pathological report diagnosed nodular retroperitoneal fibrosis associated with IgG4; Ki67-positive in germinal centers (5%) and IgG4-positive (40 plasma cells in 3 fields of 40x) by immunohistochemistry...
July 19, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28733656/efficacy-of-cyclophosphamide-treatment-for-immunoglobulin-g4-related-disease-with-addition-of-glucocorticoids
#3
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/28725444/post-tuberculous-fibrosing-mediastinitis-a-review-of-the-literature
#4
REVIEW
Zhe Wu, Hannah Jarvis, Luke S Howard, Corrina Wright, Onn Min Kon
Fibrosing mediastinitis (FM) is a rare disease where there is thickening of the fibrous tissue in the mediastinum. While histoplasmosis is the the most common recognised cause, the link with tuberculosis (TB) has been rarely documented. We review the link between TB and FM, and describe a case of probable TB-related FM.Our case is of a 74-year-old man who presented with breathlessness 3 years after fully treated TB. Scans revealed a calcified soft tissue mass within the mediastinum, and a diagnosis of fibrosing mediastinitis resulting in pulmonary hypertension was made...
2017: BMJ Open Respiratory Research
https://www.readbyqxmd.com/read/28723905/evolution-of-anti-trypanosoma-cruzi-antibody-production-in-patients-with-chronic-chagas-disease-correlation-between-antibody-titers-and-development-of-cardiac-disease-severity
#5
Ingebourg Georg, Alejandro Marcel Hasslocher-Moreno, Sergio Salles Xavier, Marcelo Teixeira de Holanda, Eric Henrique Roma, Maria da Gloria Bonecini-Almeida
Chagas disease is one of the most important endemic infections in Latin America affecting around 6-7 million people. About 30-50% of patients develop the cardiac form of the disease, which can lead to severe cardiac dysfunction and death. In this scenario, the identification of immunological markers of disease progression would be a valuable tool for early treatment and reduction of death rates. In this observational study, the production of anti-Trypanosoma cruzi antibodies through a retrospective longitudinal follow-up in chronic Chagas disease patients´ cohort and its correlation with disease progression and heart commitment was evaluated...
July 19, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28722605/characterizing-reactivity-to-onchocerca-volvulus-antigens-in-multiplex-bead-assays
#6
Karla R Feeser, Vitaliano Cama, Jeffrey W Priest, Elizabeth A Thiele, Ryan E Wiegand, Thomson Lakwo, Sendeaw M Feleke, Paul T Cantey
Multiplex bead assays (MBAs) may provide a powerful integrated tool for monitoring, evaluation, and post-elimination surveillance of onchocerciasis and co-endemic diseases; however, the specificity and sensitivity of Onchocerca volvulus antigens have not been characterized within this context. An MBA was developed to evaluate three antigens (OV-16, OV-17, and OV-33) for onchocerciasis. Receiver operating characteristics (ROC) analyses were used to characterize antigen performance using a panel of 610 specimens: 109 O...
July 3, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28720475/a-case-of-igg4-related-disease-presenting-with-multiple-gastric-submucosal-tumors
#7
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
#8
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
#9
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
#10
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...
July 14, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28710538/-manifestations-of-autoimmune-disorders-in-otorhinolaryngology-classical-symptoms-and-diagnostic-approach
#11
B Hofauer, A Chaker, K Thürmel, A Knopf
Manifestations of autoimmune disorders represent a diagnostic challenge. Many autoimmune disorders show typical manifestations in the ear, nose and throat and should be taken into account in the differential diagnosis. Sjogren's syndrome, various vasculitides and sarcoidosis represent the most common autoimmune disorders in otorhinolaryngology, but IgG4-related diseases, relapsing polychondritis and autoimmune disorders of the mucosa are also observed. Autoimmune diseases can lead to sensorineural deficits...
July 14, 2017: HNO
https://www.readbyqxmd.com/read/28708993/il-4r%C3%AE-inhibitor-for-atopic-disease
#12
Howard Y Chang, Kari C Nadeau
Dupilumab is a fully human IgG4 monoclonal antibody directed against the IL-4Rα subunit of IL-4 and IL-13 receptors. It blocks the signaling pathways of IL-4 and IL-13, key cytokines that drive type 2 inflammatory response. In March 2017, dupilumab was approved for use in the treatment of atopic dermatitis (eczema). To view this Bench to Bedside, open or download the PDF.
July 13, 2017: Cell
https://www.readbyqxmd.com/read/28708133/autoantibodies-in-chronic-inflammatory-neuropathies-diagnostic-and-therapeutic-implications
#13
REVIEW
Luis Querol, Jérôme Devaux, Ricard Rojas-Garcia, Isabel Illa
The chronic inflammatory neuropathies (CINs) are rare, very disabling autoimmune disorders that generally respond well to immune therapies such as intravenous immunoglobulin (IVIg). The most common forms of CIN are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, and polyneuropathy associated with monoclonal gammopathy of unknown significance. The field of CIN has undergone a major advance with the identification of IgG4 autoantibodies directed against paranodal proteins in patients with CIDP...
July 14, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28701054/diagnostic-and-treatment-workup-for-igg4-related-disease
#14
Mary Abraham, Arezou Khosroshahi
IgG4-Related Disease is a newly recognized condition which is increasingly diagnosed by practitioners due to improvement in clinical awareness. Men and women have been found to be affected by this disease in various organs, more commonly with involvement of the salivary and lacrimal glands as well as pancreas and liver. Areas Covered: The diagnosis and management of this condition remain challenging as biomarkers and therapies are being investigated. Hallmark features on histology are still the gold standard for confirmation of diagnosis, whereas serum IgG4 level has been shown to be neither necessary nor sufficient for the diagnosis...
July 12, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28700400/treatment-of-pediatric-igg4-related-orbital-disease-with-tnf-%C3%AE-inhibitor
#15
Sanjai Jalaj, Kristen Dunbar, Ashley Campbell, Michael Kazim
The authors describe a 9-year-old female who presented with swelling, proptosis, and tenderness of the right upper eyelid and MRI imaging demonstrating right lacrimal gland enlargement. After failing treatment with corticosteroids, the patient underwent a biopsy that was consistent with IgG4-related orbital disease. She was subsequently successfully treated with adalimumab (TNF-α inhibitor). This is the first case report of the successful use of a TNF-α inhibitor for the treatment of IgG4-related orbital disease in a child...
July 10, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28692416/immunoglobulin-g-n-glycans-as-potential-postgenomic-biomarkers-for-hypertension-in-the-kazakh-population
#16
Qing Gao, Mamatyusupu Dolikun, Jerko Štambuk, Hao Wang, Feifei Zhao, Nizam Yiliham, Youxin Wang, Irena Trbojević-Akmačić, Jie Zhang, Honghong Fang, Yang Sun, Hongli Peng, Zhongyao Zhao, Di Liu, Jiaonan Liu, Qihuan Li, Qi Sun, Lijuan Wu, Gordan Lauc, Wei Wang, Manshu Song
Next-generation (postgenomic) biomarkers from the nascent field of glycomics now offer fresh vistas for innovation in chronic disease biomarkers and system diagnostics in clinical medicine. Our previous work has shown an association between hypertension and immunoglobulin G (IgG) glycome composition, suggesting that individual variation in N-glycosylation of IgG might contribute to hypertension pathogenesis. The present study examined, for the first time to the best of our knowledge, the IgG N-glycans as potential biomarkers for hypertension in the Kazakh population...
July 2017: Omics: a Journal of Integrative Biology
https://www.readbyqxmd.com/read/28681251/igg4-related-disease-a-complex-under-diagnosed-clinical-entity
#17
REVIEW
Sujani Yadlapati, Elijah Verheyen, Petros Efthimiou
IgG4-related disease (IgG4-RD) encompasses a spectrum of complex fibro-inflammatory disorders which are often under diagnosed due to unfamiliarity by clinicians. A challenging multitude of clinical manifestations makes the diagnosis cumbersome. The primary clinical feature in IgG4-RD entails a tumor-like presentation coupled with tissue-destructive lesions. Histopathological findings include lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. These findings, in combination with elevated serum immunoglobulin G4 levels, are diagnostic in the setting of single- or multi-organ involvement...
July 5, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28679994/multicentric-castleman-s-disease-with-multiple-hepatic-mass-lesions-mimicking-malignant-liver-tumors
#18
Toshimitsu Ueki, Masaru Nasuno, Hiroko Kaiume, Yuki Hiroshima, Masahiko Sumi, Masahide Watanabe, Dai Inoue, Yasufumi Masaki, Yasuharu Sato, Masaru Kojima, Hikaru Kobayashi
Multicentric Castleman's disease (MCD) is a rare, non-malignant lymphoproliferative disorder. We report a case of MCD with multiple liver masses. A 26-year-old woman presented with asymptomatic anemia and hypoalbuminemia. Laboratory tests detected high CRP levels and findings indicative of polyclonal gammopathy. Abdominal CT revealed multiple hepatic large masses (≤10 cm) and partial calcification in the right lobe. Multiple enlarged lymph nodes were also identified in the cardiophrenic angle and porta hepatis...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28674044/pla2r-and-thsd7a-disparate-paths-to-the-same-disease
#19
REVIEW
Laurence H Beck
The phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) are the two major autoantigens in primary membranous nephropathy (MN), and define two molecular subclasses of this disease. Both proteins are large transmembrane glycoproteins expressed by the podocyte, and both induce IgG4-predominant humoral immune responses that produce circulating autoantibodies that can be used clinically for diagnostic and monitoring purposes. The biologic roles of these proteins remain speculative, although several features of THSD7A suggest a role in adhesion...
July 3, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28643686/-pachymeningitis-associated-with-igg4-disease
#20
Dan Zimelewicz Oberman, Carolina Cuello Oderiz, Matteo Baccanelli, Silvia Christiansen, María Cristina Zurrú
Hypertrophic pachymeningitis is an infrequent disorder. It can be idiopathic or secondary to infectious, autoimmune or neoplastic disease. The recently described 〈IgG4-related disease〉 could be the origin of many cases considered cryptogenic. We present the case of a 60-year-old man, with a history of headache and episcleritis in both eyes, with partial response to corticoid therapy. The brain MR study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left temporal and occipital lobes to ipsilateral tentorium...
2017: Medicina
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