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IgG4-related disease

Mitsuhiro Akiyama, Tsutomu Takeuchi
IgG4-related disease is a heterogeneous immune-mediated fibroinflammatory condition that can affect every single organ. This disease is more prevalent in the elderly (the mean age of patients is above 60 years) and the prevalence rate is estimated to be over 4.6 per 100,000 population. Before making a diagnosis, the exclusion of malignancies, lymphoma, anti-neutrophil cytoplasmic antibody-associated vasculitis, multicentric Castleman disease, and other mimickers is crucial for appropriate treatment. Broad management guidelines have been published emphasizing the need for prompt treatment and the use of glucocorticoids as first-line drug therapy for induction of remission...
March 15, 2018: Drugs & Aging
Kenji Nishida, Yuka Gion, Mai Takeuchi, Takehiro Tanaka, Tatsuki R Kataoka, Tadashi Yoshino, Yasuharu Sato
Immunoglobulin G4 (IgG4)-related disease is characterized by elevated serum IgG4 levels and increased numbers of IgG4-positive cells. However, its pathogenesis is not fully understood. We previously suggested that mast cells may play an important role in IgG4-related disease. In this study, we confirmed the characteristics of mast cells in IgG4-related lymphadenopathy by using immunohistochemistry and dual immunofluorescence. We analyzed 23 cases of IgG4-related lymphadenopathy and compared them with 23 cases of non-specific lymphoid hyperplasia...
March 15, 2018: Scientific Reports
Yasuhiro Kinugawa, Takeshi Uehara, Kazuyuki Matsuda, Yukihiro Kobayashi, Tomoyuki Nakajima, Hideaki Hamano, Shigeyuki Kawa, Kayoko Higuchi, Noriko Hosaka, Satoshi Shiozawa, Hiroki Ishigame, Toshitsugu Nakamura, Yasuhiro Maruyama, Koh Nakazawa, Masato Nakaguro, Kenji Sano, Hiroyoshi Ota
The relationship between methylation abnormality and autoimmune pancreatitis (AIP)-a representative IgG4-related disease-has not yet been elucidated. We identified SKI might have a significant methylation abnormality in AIP through methylation array analysis using the Illumina Infinium Human Methylation 450K BeadChip array, and investigated the relationship of SKI with AIP clinicopathological features. The methylation rate of SKI was assessed by quantitative SYBR green methylation-specific PCR, and the degree of SKI expression in tissue specimens was assessed by immunohistochemistry in 10 AIP cases, 14 cases of obstructive pancreatitis area in pancreatic ductal adenocarcinoma (PDA) without a history of AIP, and 9 normal pancreas (NP) cases...
March 6, 2018: Pathology, Research and Practice
Atsushi Nagayasu, Satoshi Kubo, Kazuhisa Nakano, Shingo Nakayamada, Shigeru Iwata, Ippei Miyagawa, Shunsuke Fukuyo, Kazuyoshi Saito, Yoshiya Tanaka
An 81-year-old man was admitted with bilateral pleural effusion. A clinical examination showed lymphocytic pleura effusion and elevated serum IgG4 levels, so that IgG4-related disease was suggested, whereas tuberculous pleurisy was suspected because of high adenosine deaminase (ADA) levels in the pleural effusion. A surgical pleural biopsy revealed that there were large numbers of IgG4-positive cells and IgG4/IgG positive cell ratio exceeded 40% in several sites. Accordingly, we diagnosed IgG4-related pleuritis and treated with the patient with glucocorticoid therapy...
March 9, 2018: Internal Medicine
Shounak Majumder, Sonmoon Mohapatra, Ryan J Lennon, Guilherme Piovezani Ramos, Neil Postier, Ferga Gleeson, Michael J Levy, Randall K Pearson, Bret T Petersen, Santhi S Vege, Suresh T Chari, Mark Topazian, Thomas E Witzig
BACKGROUND & AIMS: Immunoglobulin G4-related disease (IgG4-RD), a multi-organ fibro-inflammatory syndrome, typically responds to steroids. However, some cases are steroid resistant, and pancreaticobiliary IgG4-RD commonly relapses after steroid withdrawal. Rituximab induces remission of IgG4-RD, but the need for and safety of maintenance rituximab treatment are unknown. We compared outcomes of patients with pancreaticobiliary IgG4-RD treated with or without maintenance rituximab therapy...
March 8, 2018: Clinical Gastroenterology and Hepatology
Kyoko Otani, Dai Inoue, Tomoo Itoh, Yoh Zen
AIMS: The diagnosis of IgG4-related disease (IgG4-RD) requires a multidisciplinary approach, in which histology plays an important role. Although a diagnosis was previously established using surgically resected specimens, there is increasing clinical demand to diagnose this systemic condition by biopsies. The present study aimed to elucidate how useful transbronchial lung biopsies (TBLBs) are for this diagnostic purpose. METHODS AND RESULTS: The study cohort consisted of 20 consecutive patients diagnosed with IgG4-RD in other organs who underwent TBLBs for potential pulmonary involvement...
March 11, 2018: Histopathology
Ken Kamata, Tomohiro Watanabe, Kosuke Minaga, Warren Strober, Masatoshi Kudo
Autoimmune pancreatitis (AIP) is a chronic fibro-inflammatory disorder of the pancreas. However, extensive clinico-pathological analyses have revealed that AIP is, in reality, a pancreatic manifestation of a newly described systemic disease known as IgG4-related disease (IgG4-RD). IgG4-RD is characterized by enhanced local and systemic IgG4 antibody (Ab) responses as well as inflammation involving multiple organs, including the pancreas, bile ducts, and salivary glands. Although mice lack the IgG4 Ab subtype, autoimmune-prone MRL/Mp mice treated with repeated injection with polyinosinic-polycytidylic acid (poly (I:C)) provide an experimental model of AIP...
February 21, 2018: Current Protocols in Immunology
Camila Cortés, Silvia Bielsa, Josep Manel Fernandez, Felip Vilardell Villellas, Blanca Gonzalez-Farré, Josep Manel Casanova
No abstract text is available yet for this article.
March 2, 2018: Acta Dermato-venereologica
Jie Chang, Wen Zhang
IgG4-related disease (IgG4-RD) is a chronic and systemic disease that can involve multiple organs. The most commonly involved organs include the salivary glands, orbital adnexal structures, paranasal sinus, thyroid, lungs, breasts, aorta, pancreas, biliary ducts, kidneys, retroperitoneum, lymph nodes, prostate, pituitary, and endocranium. Due to increased disease research, several new site-specific nuances of IgG4-RD have been described. The authors have reviewed the recent literature and briefly summarize the infrequent organ involvement of IgG4-RD...
March 3, 2018: Clinical Rheumatology
Emanuel Della-Torre, Emanuele Bozzalla-Cassione, Clara Sciorati, Eliana Ruggiero, Marco Lanzillotta, Silvia Bonfiglio, Hamid Mattoo, Cory A Perugino, Enrica Bozzolo, Lucrezia Rovati, Paolo Giorgio Arcidiacono, Gianpaolo Balzano, Dejan Lazarevic, Chiara Bonini, Massimo Falconi, John H Stone, Lorenzo Dagna, Shiv Pillai, Angelo A Manfredi
OBJECTIVE: An unconventional population of CD4+ SLAMF7+ cytotoxic TEM cells (CD4+ CTLs) has been linked causally to IgG4-related disease (IgG4-RD). Glucocorticoids represent the first line therapeutic approach in patients with IgG4-RD but their mechanism of action in this specific condition remains unknown. Here we describe the impact of glucocorticoids on CD4+ CTLs in IgG4-RD. METHODS: CD8α, granzyme A, perforin, and SLAMF7 expression within the effector/memory compartment of CD45RO (TEM ) and CD45RA (TEMRA ) CD4+ T cells was quantified by flow cytometry in 18 active IgG4-RD patients at baseline and after 6 months of glucocorticoid treatment...
March 2, 2018: Arthritis & Rheumatology
Yayoi Shimazu, Tatsuki Uchiyama, Chisaki Mizumoto, Tomoharu Takeoka, Masaaki Tsuji, Kenjiro Tomo, Koji Takaori, Naoki Sakai, Tomoko Okuno, Tatsuharu Ohno
IgG4-related disease (IgG4RD) is a multi-organ disorder characterized by an elevated serum IgG4 level and IgG4-positive plasma cell infiltration of the affected organs, accompanied by tissue fibrosis and sclerosis. Although it can affect any organ, to our knowledge, no cases involving concurrent autoimmune neutropenia and thrombocytopenia have been reported. A 62-year-old man visited our hospital and was diagnosed with IgG4RD accompanied by interstitial pneumonitis, lymphadenopathy, and interstitial nephritis...
February 28, 2018: Internal Medicine
Toshio Doi, Tatsumi Moriya, Yui Fujita, Naoto Minagawa, Masaru Usami, Tomoko Sasaki, Hideharu Abe, Seiji Kishi, Taichi Murakami, Motoshi Ouchi, Go Ichien, Keiichi Yamamoto, Hiroki Ikeda, Yasuhiko Koezuka, Norimichi Takamatsu, Kenji Shima, Michael Mauer, Kojiro Nagai, Tatsuya Tominaga
Diabetic nephropathy (DN) is the major cause of end-stage kidney disease but early biomarkers of DN risk are limited. Herein we examine urinary immunoglobulin G4 (IgG4) and Smad1 as additional early DN biomarkers. 815 patients with type 2 diabetes mellitus were recruited; 554 patients fulfilled the criteria of more than 60 ml/min of estimated glomerular filtration rate (eGFR) and no macroalbuminuria at baseline with followed-up for 5 years. Patients without macroalbuminuria were also recruited for renal biopsies...
February 28, 2018: Diabetes
Prachi Dua, Roman Shinder, Derek B Laskar, Douglas R Lazzaro, Allison E Rizzuti
Purpose: To report a case of hypertrophic herpes simplex virus (HSV) of the eyelid and cornea masquerading as IgG4-related disease. Observations: A 37-year old African American female with a past medical history of human immunodeficiency virus (HIV) on highly active antiretroviral therapy (HAART) and a recent history of treated genital herpes, presented with an ulcerative lesion of the left upper and lower eyelids, and severe ocular inflammation with symblepharon...
March 2018: American Journal of Ophthalmology Case Reports
Kyoko Otani, Dai Inoue, Kohei Fujikura, Takahiro Komori, Shiho Abe-Suzuki, Takuma Tajiri, Tomoo Itoh, Yoh Zen
The present study aimed to compare clinicopathologic features between idiopathic multicentric Castleman's disease (n=22) and IgG4-related disease (n=26). Histology was analyzed using lymph node and lung biopsies. The expression of IL-6 mRNA in tissue was also examined by in situ hybridization and real-time PCR. Patients with idiopathic multicentric Castleman's disease were significantly younger than those with IgG4-related disease (p<0.001). Splenomegaly was observed in only idiopathic multicentric Castleman's disease (p=0...
January 23, 2018: Oncotarget
Jaspreet Singh Sangha Brar, Saurav Gupta, Salahudeen M Haja Mohideen, Lishya Liauw, Narayan Lath
The association between immunoglobulin IgG4 and autoimmune pancreatitis was first shown in 2001. Since then many previously established fibrosclerotic diseases demonstrating synchronous or metachronous multiorgan involvement have been included within the ambit of IgG4-related disease. Diagnostic criteria have been proposed involving 1) serum IgG4 level elevated beyond 135 mg/dL, 2) IgG4+ to IgG+ plasma cell ratio >40% and >10 IgG4+ cells per high power field of biopsy sample and 3) a constellation of imaging features which involve a variety of organ systems...
February 21, 2018: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
Jianchun Xiao, Peiran Xu, Binglu Li, Tao Hong, Wei Liu, Xiaodong He, Chaoji Zheng, Yupei Zhao
Immunoglobulin (Ig)G4-associated cholangitis (IAC) is one of the common organ manifestations of IgG4-related systemic disease (ISD). IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Diagnosis is based on a combination of clinical, biochemical, radiological, and histological findings.To study the clinical presentation of and treatment strategy for IAC, we reviewed clinical, serologic, and imaging characteristics, as well as treatment response, in 39 patients with IAC...
February 2018: Medicine (Baltimore)
Alexandra Forestier, David Buob, Tristan Mirault, Philippe Puech, Viviane Gnemmi, David Launay, Eric Hachulla, Pierre-Yves Hatron, Marc Lambert
OBJECTIVES: Retroperitoneal fibrosis (RPF) is a rare disease, with unknown aetiology (idiopathic RPF: iRPF) in two-thirds of cases. A subset of iRPF may be a manifestation of IgG4-related disease (IgG4-RD). Thus, recognition of IgG4-RD-RPF is crucial to optimise patient's care with iRPF. The current study aimed to examine imaging specific patterns, which could help differentiate between IgG4-RD-RPF and iRPF, and thus skip performing biopsies. METHODS: This analysis included patients with iRPF and a retroperitoneal biopsy at the Lille University Hospital, France...
February 14, 2018: Clinical and Experimental Rheumatology
Mitsuhiro Akiyama, Takanori Sasaki, Yuko Kaneko, Hidekata Yasuoka, Katsuya Suzuki, Kunihiro Yamaoka, Tsutomu Takeuchi
OBJECTIVES: To identify biomarkers for disease activity in IgG4-related disease (IgG4-RD) and primary Sjögren's syndrome (pSS). METHODS: Forty-three consecutive treatment-naïve patients with IgG4-RD, 62 patients with pSS, and 5 patients with sicca syndrome were enrolled. IgG4-RD and pSS disease activity was assessed based on the IgG4-RD responder index (IgG4-RD RI) and EULAR Sjögren's Syndrome Disease Activity Index (ES- SDAI), respectively. The associations of biomarkers with disease activity were examined...
February 15, 2018: Clinical and Experimental Rheumatology
Kazushige Uchida, Kazuichi Okazaki
In 1995, Yoshida and colleagues proposed the concept of "autoimmune pancreatitis" (AIP), which has recently been recognized as a new pancreatic inflammatory disease. Recent studies have suggested the existence of two subtypes of AIP: type 1, which involves immunoglobulin G4 (IgG4) and is the pancreatic manifestation of IgG4-related disease (IgG4-RD); and type 2, which is characterized by granulocytic epithelial lesions. Type 2 AIP is thought to be rare in Japan. Type 1 AIP is characterized by increased serum IgG4 concentrations, lymphoplasmacytic infiltrations, storiform fibrosis, and obliterative phlebitis...
February 19, 2018: Journal of Gastroenterology
Xiao Xiao, Min Lian, Weici Zhang, M Eric Gershwin, Xiong Ma
IgG4-related disease (IgG4-RD), which usually occurs in middle-aged and elderly men, is a newly recognized fibroinflammatory condition characterized by swelling and sclerosis of involved organs, increased IgG4-positive plasma cell infiltration in lesions, and elevated IgG4 concentration in serum. Despite growing interest in the research, the pathophysiological mechanism remains elusive. Most IgG4-RD patients respond well to steroid therapy initially, but recurrent and refractory cases are common, especially in advanced fibrotic stage...
February 19, 2018: Clinical Reviews in Allergy & Immunology
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