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IgG4- related diseases

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https://www.readbyqxmd.com/read/28231569/biomarkers-in-autoimmune-salivary-gland-disorders-a-review
#1
Benedikt Hofauer, Klaus Thuermel, Constanze Gahleitner, Andreas Knopf
Salivary glands are frequent sites of manifestations of autoimmune disorders in the head and neck. Sjögren syndrome, sarcoidosis, granulomatosis with polyangiitis, and IgG4-related sialadenitis represent the most important autoimmune salivary gland disorders. Due to the lack of specific symptoms, diagnosis of these conditions remains a challenge. Diagnosis is usually based on classification criteria involving clinical tests, histopathological evaluation, and serological examinations. Depending on the disease, biomarkers are of different value and have to be interpreted carefully...
February 24, 2017: ORL; Journal for Oto-rhino-laryngology and its related Specialties
https://www.readbyqxmd.com/read/28228909/igg4-related-disease-in-an-adolescent-with-radiologic-pathologic-correlation
#2
Renato Cesar Ferreira da Silva, Scott M Lieberman, Henry T Hoffman, Bruno Policeni, Amani Bashir, Richard J H Smith, T Shawn Sato
Immunoglobulin G4-related disease (IgG4RD) is an immune-mediated condition characterized by lymphoplasmacytic infiltrates and fibrosis of affected organs. IgG4RD may affect many different organs either individually or together in a multiorgan condition and, thus, incorporates a wide range of fibroinflammatory phenotypes with shared pathologic features. Although IgG4RD most commonly occurs in late adulthood, it may affect children and adolescents. Only one case of IgG4RD presenting as isolated submandibular gland involvement has been reported in the pediatric population...
March 2017: Radiology case reports
https://www.readbyqxmd.com/read/28225291/-a-contribution-to-the-differential-diagnostics-of-sclerosing-cholangitides
#3
Martin Blaho, Petr Dítě, Martina Bojková, Martin Rydlo, Tomáš Kupka, Pavel Svoboda, Pavel Klvaňa, Arnošt Martínek
Sclerosing cholangitides represent a group of chronic biliary obstructive diseases which include primary sclerosing cholangitis (PSC), IgG4 associated sclerosing cholangitis (IgG4-SC) and secondary sclerosing cholangitis (SSC). The manifestations of the diseases are similar, but their asymptomatic course is also frequent. IgG4-SC belongs to the group of IgG4 associated diseases and it is the most frequently related to type 1 autoimmune pancreatitis. Diagnosing of IgG4-SC is based on typical histopathological images, shape changes revealed by diagnostic imaging, serological tests, concurrent impairment of other organs and response to therapy, where IgG4-SC responds well to treatment with corticoids, whereas the only possibility for the remaining units is endoscopic intervention or liver transplantation...
2017: Vnitr̆ní Lékar̆ství
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
#4
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/28222449/characterization-of-cutaneous-plasmacytosis-at-different-disease-stages
#5
Pa-Fan Hsiao, Yu-Hung Wu
BACKGROUND/AIMS: Cutaneous plasmacytosis is rare and still not well understood. A retrospective study was made of 9 Chinese patients with 1- to 15-year histories of biopsy-proven cutaneous plasmacytosis diagnosed between 2003 and 2015. METHODS: Patient records and archival photographs helped establish the pattern and duration of skin lesions, and skin biopsy specimens provided additional data. RESULTS: The mean age at diagnosis was 46.4 years...
February 22, 2017: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/28219566/immunoglobulin-g4-related-periaortitis-involving-the-aortic-arch-mimicking-a-mediastinal-tumor
#6
Min Jae Cha, Semin Chong, Yang Soo Kim, Byungjoon Park, Ja Hee Seo, Eun Sung Lee
Immunoglobulin G4 (IgG4)-related sclerosing disease is a recently proposed systemic fibroinflammatory disease entity, with periaortitis as a representative manifestation in the cardiovascular system. We present a case of IgG4-related periaortitis involving the aortic arch and proximal great vessels, mimicking an anterior mediastinal tumor on computed tomography. In patients with a soft tissue mass along the aortic wall, IgG4-related periaortitis should be included as a differential diagnosis and timely serologic investigation should be performed to prevent unnecessary surgery, because the disease shows a remarkable response to corticosteroids...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28219556/igg4-related-disease-causing-rapid-evolution-of-a-severe-aortic-valvular-stenosis
#7
Samuel Bruls, Audrey Courtois, Philippe Delvenne, Roland Hustinx, Michel Moutschen, Laurence De Leval, Jean-Olivier Defraigne, Natzi Sakalihasan
Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recognized emerging entity characterized by chronic fibroinflammation that can affect every organ but rarely affects the cardiovascular system. We report a rare case of IgG4-RSD involving an aortic valve that resulted in rapid progression of an aortic valvular stenosis and was successfully treated by aortic valve replacement and corticosteroids.
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28215485/association-of-igg4-response-and-autoimmune-pancreatitis-with-intraductal-papillary-mucinous-neoplasms
#8
Amin A Hedayat, Mikhail Lisovsky, Arief A Suriawinata, Daniel S Longnecker
OBJECTIVES: Concurrent intraductal papillary-mucinous neoplasm (IPMN) and autoimmune pancreatitis (AIP) was observed in a patient (index case) at our institution. Cases of coincidental IPMN and type 1 AIP and concurrent ductal adenocarcinoma (PDAC) and AIP have been previously reported. In this study we evaluate the hypothesis that IPMN elicits an IgG4 response. METHODS: Twenty-one pancreases (including the index case) with IPMN resected at our institution were studied...
February 8, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
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
#9
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
#10
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/28202062/isolated-igg4-related-sclerosing-cholangitis-misdiagnosed-as-malignancy-in-an-area-with-endemic-cholangiocarcinoma-a-case-report
#11
Narongsak Rungsakulkij, Pattana Sornmayura, Penampai Tannaphai
BACKGROUND: The most common cause of perihilar obstruction is cholangiocarcinoma, especially in Thailand. Benign perihilar stricture represents less than 20% of cases. IgG4-related disease and IgG4-related sclerosing cholangitis, however, have been receiving increased recognition. Isolated IgG4-related sclerosing cholangitis is less common. The preoperative diagnosis of IgG4-related sclerosing cholangitis without pancreatic involvement is very difficult because the clinical presentation and preoperative evaluation are extremely difficult to distinguish from perihilar cholangiocarcinoma...
February 15, 2017: BMC Surgery
https://www.readbyqxmd.com/read/28197739/diagnosis-and-treatment-of-igg4-related-disease
#12
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...
February 15, 2017: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/28197205/from-pathogenesis-clinical-manifestation-and-diagnosis-to-treatment-an-overview-on-autoimmune-pancreatitis
#13
REVIEW
Ou Cai, Shiyun Tan
Autoimmune pancreatitis (AIP) is a special type of chronic pancreatitis which is autoimmune mediated. The international consensus diagnostic criteria (ICDC) 2011 proposed two types of AIP: type I is associated with histological pattern of lymphoplasmacytic sclerosing pancreatitis (LPSP), characterized by serum IgG4 elevation, whereas type 2 is named idiopathic duct-centric pancreatitis (IDCP), with granulocytic epithelial lesion (GEL) and immunoglobulin G4 (IgG4) negative. The pathogenic mechanism is unclear now; based on genetic factors, disease specific or related antigens, innate and adaptive immunity may be involved...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28196014/methylation-of-tumor-suppressor-genes-in-autoimmune-pancreatitis
#14
Yasuhiro Kinugawa, Takeshi Uehara, Kenji Sano, Kazuyuki Matsuda, Yasuhiro Maruyama, Yukihiro Kobayashi, Tomoyuki Nakajima, Hideaki Hamano, Shigeyuki Kawa, Kayoko Higuchi, Noriko Hosaka, Satoshi Shiozawa, Hiroki Ishigame, Hiroyoshi Ota
OBJECTIVES: Autoimmune pancreatitis (AIP) is a representative IgG4-related and inflammatory disease of unknown etiology. To clarify mechanisms of carcinogenesis resulting from AIP, we focused on methylation abnormalities and KRAS mutations in AIP. METHODS: Six tumor suppressor genes (NPTX2, Cyclin D2, FOXE1, TFPI2, ppENK, and p16) that exhibited hypermethylation in pancreatic carcinoma were selected for quantitative SYBR green methylation-specific polymerase chain reaction in 10 AIP specimens, 10 pancreatic adenocarcinoma cases without history of AIP containing carcinoma areas (CAs) and noncarcinoma areas (NCAs), and 11 normal pancreas (NP) samples...
February 14, 2017: Pancreas
https://www.readbyqxmd.com/read/28183334/circulating-plasmablasts-plasma-cells-a-potential-biomarker-for-igg4-related-disease
#15
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
#16
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
#17
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
#18
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
#19
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
https://www.readbyqxmd.com/read/28134406/-the-ormond-s-disease-an-intriguing-obstructive-nephrouropathy
#20
Rocco Manganelli, Serena Manganelli, Salvatore Iannaccone, Walter De Simone
Idiopathic retroperitoneal fibrosis also known as Ormonds disease is a rare disorder characterized by the development of fibrotic tissue in the retroperitoneum involving the abdominal aorta and iliac arteries, ureters and the inferior vena cava. The aberrant tissue may compress ureters leading to obstructive nephrouropathy and renal failure, which are the most common clinical manifestations of this condition. The nephrologist is often consulted to make differential diagnosis for acute renal failure and obstructive uropathy...
November 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
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