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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/28710703/igg4-related-disease-involving-polyserous-effusions-with-elevated-serum-interleukin-6-levels-a-case-report-and-literature-review
#1
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/28701054/diagnostic-and-treatment-workup-for-igg4-related-disease
#2
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/28681251/igg4-related-disease-a-complex-under-diagnosed-clinical-entity
#3
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/28639844/diagnostic-sensitivity-of-cutoff-values-of-igg4-positive-plasma-cell-number-and-igg4-positive-cd138-positive-cell-ratio-in-typical-multiple-lesions-of-patients-with-igg4-related-disease
#4
Ichiro Mizushima, Kazunori Yamada, Kenichi Harada, Shoko Matsui, Takako Saeki, Satoru Kondo, Masayuki Takahira, Yuko Waseda, Yasuhito Hamaguchi, Hiroshi Fujii, Masakazu Yamagishi, Mitsuhiro Kawano
OBJECTIVES: This study aimed to investigate the diagnostic sensitivity of the cutoff values of IgG4-positive plasma cell (PC) number and IgG4-positive/CD138-positive cell ratio proposed by the International consensus statement (ICS) on the pathology of IgG4-related disease (IgG4-RD) in typical multiple lesions of patients with IgG4-RD. METHODS: We evaluated IgG4-positive PC number and IgG4-positive/CD138-positive cell ratio in 39 samples from 18 IgG4-RD patients having more than two typical lesions of IgG4-RD...
June 22, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28621822/cdna-microarray-analysis-identifies-nr4a2-as-a-novel-molecule-involved-in-the-pathogenesis-of-sj%C3%A3-gren-s-syndrome
#5
Hiroyuki Takahashi, Hiroto Tsuboi, Hiromitsu Asashima, Tomoya Hirota, Yuya Kondo, Masafumi Moriyama, Isao Matsumoto, Seiji Nakamura, Takayuki Sumida
OBJECTIVE: To examine genes expressed specifically in labial salivary glands (LSGs) of patients with Sjögren's syndrome (SS) in comparison with those of patients with IgG4-related disease (IgG4-RD) and to identify the genes involved in the pathogenesis of SS. METHODS: Gene expression in LSGs of SS patients, IgG4-RD patients and healthy controls (HC) was analyzed by cDNA microarray. Quantitative PCR (qPCR) was used to validate the up-regulation of differentially expressed genes (DEGs) in SS...
June 16, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28614225/immunoglobulin-g4-related-disease-preceded-by-lung-involvement-a-case-report
#6
Miki Abo, Hazuki Takato, Satoshi Watanabe, Kazumasa Kase, Tamami Sakai, Hayato Koba, Johsuke Hara, Takashi Sone, Hideharu Kimura, Kazuo Kasahara
RATIONALE: Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition involving various organs and vessels including the pancreas, bile duct, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, meninges, and aorta. Recently, some cases of IgG4-RD have been reported, in which only pulmonary lesions were present. It is not known whether IgG4-RD can be diagnosed on the basis of pulmonary lesions only, because increases in serum IgG4 levels and infiltration of IgG4-positive plasma cells into the lung tissue also occur in other inflammatory conditions...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28614220/rituximab-was-used-to-treat-recurrent-igg4-related-hypophysitis-with-ophthalmopathy-as-the-initial-presentation-a-case-report-and-literature-review
#7
REVIEW
Wei-Jun Gu, Qian Zhang, Jian Zhu, Jie Li, Shi-Hui Wei, Yi-Ming Mu
RATIONALE: Immunoglobulin G4 (IgG4)-related hypophysitis is a type of IgG4-related disease (IgG4-RD), which is characterized by plasma cells infiltration in the pituitary causing functional changes and (or) space-occupying effect in the pituitary. IgG4-related hypophysitis is sensitive to hormone therapy in most patients, but recurrence is very likely. PATIENT CONCERNS: Here, we report a 57-year-old male patient with bilateral eye redness as the initial presentation...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28607901/igg4-related-disease-igg4-rd-presenting-as-a-mass-in-the-carotid-triangle-masquerading-paraganglioma
#8
T Ramadass, V Balaji, S K J Sheba, K S Vali Ahmed, Raees Abdurahiman
IgG4-related disease (IgG4-RD) which is a protein disorder presented as a mass in the right carotid triangle in a 30 year male patient, who underwent battery of tests is described. The radiologist opined the mass as paraganglioma and the spindle shaped character of the mass also suggested neurogenic tumor in differential diagnosis. Reference to vascular surgeon also opined the same, and adviced for incisional biopsy. Histopathology report suggested IgG4-RD and immunochemistry confirmed the final diagnosis...
June 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28596203/surgical-management-of-isolated-mesenteric-autoimmune-disease-addressing-the-spectrum-of-igg4-related-disease-and-sclerosing-mesenteritis
#9
Alissa Greenbaum, Nour Yadak, Steven Perez, Ashwani Rajput
IgG4-related disease (IgG4-RD) is a rare form of autoimmune sclerosing disease, characterised by elevated serum IgG4 and tissue IgG4 levels, specific histopathological findings, multiorgan involvement and adequate response to glucocorticoid treatment. The low incidence and the heterogeneous nature of the disease has made consensus on diagnostic criteria for IgG4-RD difficult. Whether sclerosing mesenteritis (SM) is considered a manifestation of IgG4-RD is strongly debated. We present the case of a patient with a history of rheumatoid arthritis who presented with a calcified abdominal mass...
June 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28593065/high-expression-of-galectin-3-in-patients-with-igg4-related-disease-a-proteomic-approach
#10
Adeeb Salah, Hajime Yoshifuji, Shinji Ito, Koji Kitagori, Kaori Kiso, Norishige Yamada, Toshiki Nakajima, Hironori Haga, Tatsuaki Tsuruyama, Aya Miyagawa-Hayashino
OBJECTIVES: Immunoglobulin G4-related disease (IgG4-RD) is a multiorgan condition manifesting itself in different forms. This study aimed to investigate protein expression profiles and to find the possible biomarker for IgG4-RD by liquid chromatography mass spectrometry (LC-MS) using tissue sections in IgG4-RD patients. METHODS: Protein expression profiles in five IgG4-related pancreatitis and three normal pancreatic samples were compared using LC-MS and were validated by quantitative real-time PCR (qRT-PCR), immunoblotting, and immunohistochemistry...
2017: Pathology Research International
https://www.readbyqxmd.com/read/28556584/efficacy-between-high-and-medium-doses-of-glucocorticoid-therapy-in-remission-induction-of-igg4-related-diseases-a-preliminary-randomized-controlled-trial
#11
Qingjun Wu, Jie Chang, Hua Chen, Yu Chen, Hongxian Yang, Yunyun Fei, Panpan Zhang, Xiaofeng Zeng, Fengchun Zhang, Wen Zhang
AIM: In order to evaluate the efficacy and safety of high versus medium doses of glucocorticoid therapy in remission induction of immunoglobulin G4-related disease (IgG4-RD), we set up a randomized controlled study. METHOD: Newly diagnosed IgG4-RD patients were randomly assigned to two groups: high doses of prednisone (0.8-1.0 mg/kg/day) and medium doses (0.5-0.6 mg/kg/day). Patients were assessed at weeks 0, 4, 12 and 24. The primary outcome was the remission rate at week 24...
May 29, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28550399/addition-of-second-line-steroid-sparing-immunosuppressants-like-mycophenolate-mofetil-improves-outcome-of-immunoglobulin-g4-related-disease-igg4-rd-a-series-from-a-tertiary-care-teaching-hospital-in-south-india
#12
Nikhil Gupta, John Mathew, Hindhumathi Mohan, Sudipta Dhar Chowdhury, Reuben Thomas Kurien, D J Christopher, Balamugesh Thangakunam, Mathew Alexander, Ajith Sivadasan, V Tamilarasi, Anna T Valson, Mahasampath Gowri, Jayakanthan Kabeerdoss, Debashish Danda
IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease. This disease may be associated with elevated serum and tissue IgG4 levels. Early treatment prevents fibrosis and organ damage. We retrospectively studied the clinicopathologic correlation and outcome of treatment in IgG4-RD. This single-center retrospective study was done using electronic records of patients subjected to assay of serum IgG4 levels in our laboratory by nephelometry. There were 473 patients with suspected IgG4-RD. Of them, 41 patients fulfilled comprehensive diagnostic criteria for IgG4-RD and 432 had diseases other than IgG4-RD...
May 26, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28544157/malignancies-in-korean-patients-with-immunoglobulin-g4-related-disease
#13
Sung Soo Ahn, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
AIM: Several studies have implicated increased risk of malignancy in patients with immunoglobulin G4-related disease (IgG4-RD). Hence, we first evaluated the risk and the type of malignancy in Korean IgG4-RD patients. METHOD: Clinical and laboratory results of patients fulfilling the comprehensive diagnostic criteria for IgG4-RD were retrospectively collected between January 2006 and November 2015. One hundred and eighteen patients were included, and 35 were classified as having definite, 83 as possible/probable IgG4-RD...
May 22, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28540606/diagnostic-performances-of-serum-igg4-concentration-and-igg4-igg-ratio-in-igg4-related-disease
#14
Chang-Sheng Xia, Chun-Hong Fan, Yan-Ying Liu
Patients with IgG4-related disease (IgG4-RD) often have elevated serum IgG4 levels. Here, we aimed to evaluate the diagnostic performances of elevated serum IgG4 concentration and IgG4/IgG ratio for IgG4-RD. We retrospectively analyzed 1381 patients subjected to serum IgG subclass testing to differentiate IgG4-RD from other diseases at Peking University People's Hospital from 2012 to 2016. This sample included 133 IgG4-RD patients and 1248 non-IgG4-RD patients. Serum IgG subclass concentrations were measured using Siemens reagents...
May 24, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28528047/sclerosing-aortic-and-coronary-arteritis-due-to-igg4-related-disease
#15
Mikael Barbu, Ulf Lindström, Claes Nordborg, Andreas Martinsson, Christian Dworeck, Anders Jeppsson
We present the case of a 55-year-old woman admitted for a coronary artery bypass operation because of three-vessel coronary artery disease based on angiographic findings and clinical symptoms. Unexpected intraoperative findings with diffuse tissue thickening of the ascending aorta and coronary arteries indicated an alternate pathogenesis rather than coronary artery atherosclerosis. Histopathologic findings and clinical evaluation could confirm IgG4-related disease (IgG4-RD). IgG4-RD is a newly recognized fibroinflammatory condition that can present in a variety of organs and is characterized by common histopathologic features...
June 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28514287/concurrent-igg4-related-tubulointerstitial-nephritis-and-igg4-myeloperoxidase-anti-neutrophil-cytoplasmic-antibody-positive-crescentic-glomerulonephritis-a-case-report
#16
Tao Su, Li Yang, Zhao Cui, Su-Xia Wang, Ming-Hui Zhao
RATIONALE: IgG4-related disease (IgG4-RD) is a newly recognized systemic disease. The typical pathological finding in the kidney is abundant IgG4-positive plasma cell infiltration with characteristic storiform fibrosis in the interstitium. Antibodies of the IgG4 subclass have been linked to certain autoimmune diseases including antiproteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) of the IgG4 subclass. Here, we report a rare case of kidney injury with concurrent typical IgG4-related tubulointerstitial nephritis and IgG4 subclass of myeloperoxidase (MPO) ANCA-positive necrotizing crescentic glomerulonephritis...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28509786/igg4-related-disease-of-the-biliary-tract-and-pancreas-clinical-and-experimental-advances
#17
Lowiek M Hubers, Ulrich Beuers
PURPOSE OF REVIEW: IgG4-related disease (IgG4-RD) is an immune-mediated disease of unknown cause. It predominantly affects the biliary tract [IgG4-associated cholangitis (IAC)] and pancreas [autoimmune pancreatitis (AIP)] of mostly elderly men. Accurate diagnostic tests are lacking. Patients benefit from predniso(lo)ne treatment. However, disease relapse is often seen. This review will address pathophysiological aspects and advances in diagnostic and therapeutic strategies. RECENT FINDINGS: The role of IgG1 and IgG4 in the pathophysiology of IgG4-RD was studied in mice which showed more intense organ damage of pancreas and salivary glands when IgG1 rather than IgG4 of patients with IgG4-RD was injected...
July 2017: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28474137/clinical-features-and-predictive-value-of-serum-inflammatory-markers-of-perivascular-involvement-in-immunoglobulin-g4-related-disease
#18
Hideya Yamamoto, Eiji Sugiyama, Masahiro Serikawa, Fuminari Tatsugami, Michiya Yokozaki, Toshiro Kitagawa, Atsushi Senoo, Yumiko Kubo Nakamoto, Kazuo Awai, Kazuaki Chayama, Yasuki Kihara
Vascular and/or perivascular involvements of sclerotic inflammation (perivasculitis) are a complication of immunoglobulin G4-related disease (IgG4-RD). We sought to examine clinical manifestations of perivasculitis by computed tomography (CT) in patients with elevated serum IgG4 levels, and then to evaluate some potential predictors of perivasculitis in definite IgG4-RD patients. From a database of patients with serum IgG4 measurements, we selected 81 patients with elevated serum IgG4 levels (≥135 mg/dl)...
May 4, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28460058/follicular-helper-t-cells-in-the-pathogenesis-of-igg4-related-disease
#19
Mitsuhiro Akiyama, Katsuya Suzuki, Hidekata Yasuoka, Yuko Kaneko, Kunihiro Yamaoka, Tsutomu Takeuchi
IgG4-related disease (IgG4-RD) is a recently recognized disease entity characterized by high serum IgG4 concentrations and infiltration of IgG4+ plasma cells with hyperplastic ectopic germinal centres at affected sites. Although the underlying immune mechanism of this disease remains unclear, T cells are abundantly present at affected sites and key players in IgG4-RD pathogenesis. The role of T cell subsets has been investigated thoroughly in this disease. Recent advances in this field have clarified the importance of T follicular helper cells...
April 28, 2017: Rheumatology
https://www.readbyqxmd.com/read/28458319/overlap-of-igg4-related-disease-and-multicentric-castleman-s-disease-in-a-patient-with-skin-lesions
#20
Hiroki Mochizuki, Manako Kato, Takakazu Higuchi, Ryosuke Koyamada, Satoru Arai, Sadamu Okada, Hikaru Eto
A 59-year-old man presented with multiple dark red erythemas with induration, anemia, and polyclonal hypergammaglobulinemia. A skin biopsy revealed the infiltration of lymphocytes and plasma cells and he was initially diagnosed with multicentric Castleman's disease (MCD). Glucocorticoid treatment was only partially effective. Four years later, the patient's bilateral lacrimal glands gradually became enlarged and a biopsy revealed dense lymphocyte and plasma cell infiltration with an IgG4+/IgG+ plasma cell ratio of 70%...
2017: Internal Medicine
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