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IgG4- related diseases ( IgG4-RD )

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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
#1
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
#2
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...
March 17, 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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
Yasuhiro Terasaki, Shinichro Ikushima, Shoko Matsui, Akira Hebisawa, Yasunori Ichimura, Shinyu Izumi, Masuo Ujita, Machiko Arita, Keisuke Tomii, Youko 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 (IgG4-L) of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion (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
https://www.readbyqxmd.com/read/28205524/interleukin-33-produced-by-m2-macrophages-and-other-immune-cells-contributes-to-th2-immune-reaction-of-igg4-related-disease
#14
Sachiko Furukawa, Masafumi Moriyama, Kensuke Miyake, Hitoshi Nakashima, Akihiko Tanaka, Takashi Maehara, Mana Iizuka-Koga, Hiroto Tsuboi, Jun-Nosuke Hayashida, Noriko Ishiguro, Masaki Yamauchi, Takayuki Sumida, Seiji Nakamura
IgG4-related disease (IgG4-RD) is characterized by elevated serum IgG4 and marked infiltration of IgG4-positive cells in multiple organs. Interleukin-33 (IL-33) is a recently described cytokine that is secreted by damaged epithelial cells, macrophages, and dendritic cells, and potently activates helper T type 2 (Th2) immune responses, which have been suggested to play a major role in IgG4 production of IgG4-RD. Here, we assessed the expression of IL-33 and related molecules in the salivary glands (SGs) of patients with IgG4-RD versus that in patients with Sjögren's syndrome (SS) and controls...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28197739/diagnosis-and-treatment-of-igg4-related-disease
#15
Terumi Kamisawa, Kazuichi Okazaki
It is critical to differentiate IgG4-related disease (IgG4-RD) from malignant tumor and similar disease of the affected organ to apply appropriate therapy and avoid unnecessary surgery. IgG4-RD is diagnosed on combination of typical radiological findings; elevation of serum IgG4 levels; histopathological findings of abundant infiltration of IgG4-positive plasma cells and lymphocytes, storiform fibrosis , and obliterative phlebitis ; association with other IgG4-related diseases; and response to steroids. Histopathological approach is particularly recommended...
2017: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/28183334/circulating-plasmablasts-plasma-cells-a-potential-biomarker-for-igg4-related-disease
#16
Wei Lin, Panpan Zhang, Hua Chen, Yu Chen, Hongxian Yang, Wenjie Zheng, Xuan Zhang, Fengxiao Zhang, Wen Zhang, Peter E Lipsky
BACKGROUND: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a multisystem fibroinflammatory disease. We previously reported that a circulating cell population expressing CD19(+)CD24(-)CD38(hi) was increased in patients with IgG4-RD. In this study, we aimed to document that this cell population represented circulating plasmablasts/plasma cells, to identify the detailed phenotype and gene expression profile of these IgG4-secreting plasmablasts/plasma cells, and to determine whether this B-cell lineage subset could be a biomarker in IgG4-related disease (IgG4-RD)...
February 10, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28166682/clonally-expanded-cytotoxic-cd4-t-cells-and-the-pathogenesis-of-igg4-related-disease
#17
Hamid Mattoo, John H Stone, Shiv Pillai
IgG4-related disease (IgG4-RD) is a systemic condition of unknown cause characterized by highly fibrotic lesions, with dense lymphoplasmacytic infiltrates containing a preponderance of IgG4-expressing plasma cells. CD4(+) T cells and B cells constitute the major inflammatory cell populations in IgG4-RD lesions. IgG4-RD patients with active, untreated disease show a marked expansion of plasmablasts in the circulation. Although the therapeutic depletion of B cells suggests a role for these cells in the disease, a direct role for B cells or IgG4 in the pathogenesis of IgG4-RD is yet to be demonstrated...
February 2017: Autoimmunity
https://www.readbyqxmd.com/read/28165852/current-approach-to-the-diagnosis-of-igg4-related-disease-combination-of-comprehensive-diagnostic-and-organ-specific-criteria
#18
Hisanori Umehara, Kazuichi Okazaki, Takuji Nakamura, Tomomi Satoh-Nakamura, Akio Nakajima, Mitsuhiro Kawano, Tsuneyo Mimori, Tsutomu Chiba
IgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criteria has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan, published comprehensive diagnostic criteria for IgG4-RD...
February 6, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28158996/igg4-positive-cell-infiltration-in-various-cardiovascular-disorders-results-from-histopathological-analysis-of-surgical-samples
#19
Ryoto Hourai, Satomi Kasashima, Koichi Sohmiya, Yohei Yamauchi, Hideki Ozawa, Yoshinobu Hirose, Yasuhiro Ogino, Takahiro Katsumata, Masahiro Daimon, Shu-Ichi Fujita, Masaaki Hoshiga, Nobukazu Ishizaka
BACKGROUND: The diagnosis of Immunoglobulin G4 (IgG4)-related disease (IgG4-RD), in general, depends on serum IgG4 concentrations and histopathological findings; therefore, diagnosis of IgG4-RD in cardiovascular organs/tissues is often difficult owing to the risk of tissue sampling. METHODS: Prevalence of IgG4-positive lymphoplasmacytic infiltration in 103 consecutive cardiovascular surgical samples from 98 patients with various cardiovascular diseases was analyzed immunohistochemically...
February 3, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28149653/igg4-related-disease-presenting-as-isolated-scleritis
#20
Eran Berkowitz, Ella Arnon, Alona Yaakobi, Yuval Cohen, Beatrice Tiosano
A rare case of IgG4-related disease (IgG4-RD) manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms...
2017: Case Reports in Ophthalmological Medicine
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