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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/28528047/sclerosing-aortic-and-coronary-arteritis-due-to-igg4-related-disease
#1
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
#2
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
#3
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...
May 13, 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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28457528/disease-associations-with-isolated-elevations-of-each-of-the-four-igg-subclasses
#7
Sarah Engelhart, Robert J Glynn, Peter H Schur
PURPOSE: Immunoglobulin G4-related disease (IgG4-RD) is a relatively newly defined disease entity that refers to a group of immune-mediated disorders that have certain histopathologic, serologic, and clinical features in common. IgG4-RD is often associated with elevated serum IgG4. The discovery of IgG4-RD highlights the scarcity of literature examining elevations in other IgG subclasses and their potential associations to disease. In this retrospective chart review study, we aim to address that gap, by exploring disease associations in patients with isolated IgG subclass elevations...
March 30, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28449462/pulmonary-function-tests-findings-and-their-diagnostic-value-in-patients-with-igg4-related-disease
#8
Lu Cao, Yi-Bing Chen, Da-Hui Zhao, Wen-Fang Shi, Song Meng, Li-Xin Xie
BACKGROUND: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can affect most organs. To date, there have been no detailed assessments of pulmonary function in patients with IgG4-RD. In this study, we investigated pulmonary function in IgG4-RD patients and evaluated the value of pulmonary function tests (PFTs) in diagnosing IgG4-related respiratory disease (IgG4-RRD). METHODS: This was a retrospective study of 17 patients with IgG4-RD...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28443233/a-case-of-immunoglobulin-g4-related-respiratory-disease-with-multiple-lung-cysts-a-case-report
#9
Hironori Mikumo, Naoki Hamada, Eiji Harada, Toyoshi Yanagihara, Saiko Ogata, Hidetake Yabuuchi, Kayo Ijichi, Koichi Takayama, Yoichi Nakanishi
A 48-year-old man was admitted for evaluation of abnormal shadows on chest radiograph. Chest computed tomography (CT) showed cysts, nodules, and cervical and axillary lymphadenopathies. Elevated serum levels of IgG4 and interleukin (IL)-6 suggested IgG4-related disease (IgG4-RD) or multicentric Castleman's disease (MCD). Histologic findings of the cervical lymph node and right lung S(6) biopsies revealed numerous IgG4-positive plasma cells. Although CT findings of the lungs were atypical for IgG4-RD, consistent histologic findings, clinical symptoms, and laboratory data made us conclude IgG4-RD...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28438752/igg4-related-disease-presenting-as-posterior-scleritis-and-vitritis-progressing-to-multifocal-orbital-involvement
#10
Gavin L Reynolds, Jonathan H Norris, Sher Aslam, Srilakshmi Sharma
IgG4-related disease (IgG4-RD) is a rare, chronic inflammatory condition that may involve nearly every organ system. Originally identified as a cause of autoimmune pancreatitis, its characteristic histological and clinical features have been found in a wide variety of inflammatory presentations, including the eye and orbit. Here we describe an example of a case of IgG4-RD initially presenting as scleritis and vitritis, with further progression to multifocal bilateral orbital involvement. Tissue biopsy of an orbital mass was highly characteristic of IgG4-RD histology and a rapid clinical response to corticosteroids was observed...
April 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28434701/upregulated-interleukins-il-6-il-10-and-il-13-in-immunoglobulin-g4-related-aortic-aneurysm-patients
#11
Satomi Kasashima, Atsuhiro Kawashima, Yoh Zen, Satoru Ozaki, Fuminori Kasashima, Masamitsu Endo, Yasushi Matsumoto, Kengo Kawakami
OBJECTIVE: Immunoglobulin (Ig) G4-related aortic aneurysms (IgG4-AAs) are a special aortic aneurysm among IgG4-related diseases (IgG4-RDs), which are inflammatory and fibrous conditions characterized by tumorous swelling of affected organs and high serum IgG4 concentrations. Recently, IgG4-RD pathogenesis was shown to be associated with T-helper-2 (Th2) and regulatory T (Treg) dominant cytokine production, such as interleukin (IL)-4, IL-10, and IL-13. IL-6 is a key proinflammatory cytokine contributing to lymphocyte and plasmacyte maturation and to atherosclerosis and aneurysm development...
April 20, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28420843/rosai-dorfman-disease-of-the-lung-overlapping-with-igg4-related-disease-the-difficulty-in-its-differential-diagnosis
#12
Mizue Hasegawa, Fumikazu Sakai, Asako Okabayashi, Hideki Katsura, Toshiko Kamata, Eitetsu Koh, Yasuo Sekine, Tamiko Takemura, Yukio Nakatani, Kenzo Hiroshima
We herein report a case of Rosai-Dorfman disease (RDD) overlapping with IgG4-related disease (IgG4-RD), which presented as diffuse interstitial lung disease with a perilymphatic pattern, followed by submandibular gland and eyelid swelling. The pathological findings of the submandibular gland biopsy specimen were indicative of IgG4-RD alone. We diagnosed the patient with RDD with overlapping IgG4-RD. However, the optimal method for differentiating between these two entities is still controversial. It is important that clinicians are aware that RDD should be included in the differential diagnoses of diffuse interstitial lung disease with a perilymphatic pattern and that RDD can overlap with IgG4-RD...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28412148/no-evidence-to-support-a-role-for-helicobacter-pylori-infection-and-plasminogen-binding-protein-in-autoimmune-pancreatitis-and-igg4-related-disease-in-a-uk-cohort
#13
Emma L Culver, Wouter L Smit, Caroline Evans, Ross Sadler, Tamsin Cargill, Mateusz Makuch, Lai-Mun Wang, Berne Ferry, Paul Klenerman, Eleanor Barnes
BACKGROUND AND OBJECTIVES: Helicobacter pylori (H.pylori) plasminogen binding protein (PBP) has been proposed as an antigen triggering autoimmune pancreatitis (AIP), the pancreatic manifestation of IgG4-related disease (IgG4-RD). We investigated exposure to H. pylori infection, cytokine response and immunological memory to H. pylori PBP in a prospective IgG4-RD cohort in the UK. METHODS: Clinical and endoscopic evidence of peptic ulceration, serological H. pylori exposure and serum IgG4 levels were obtained in 55 IgG4-RD patients and 52 disease controls (DC) with autoimmune or inflammatory conditions with an elevated serum IgG4...
April 5, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/28375417/igg4-aortitis-a-case-report
#14
Shivali Marketkar, Mark LeGolvan
IgG4 aortitis is one of the entities seen in the spectrum of IgG4-related disease (IgG4-RD). It is characterized by serologic (elevated serum IgG4) and histologic features including a lymphoplasmacytic infiltrate with increased numbers of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Some studies have described a correlation between infections and IgG4 aortitis. We describe a patient with an aneurysm of the infrarenal descending abdominal aorta with features of IgG4-RD, as well as culture evidence of Streptococcus sanguis...
April 3, 2017: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/28374231/neurological-manifestations-of-igg4-related-disease
#15
REVIEW
Bernardo Baptista, Alina Casian, Harsha Gunawardena, David D'Cruz, Claire M Rice
IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised-hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required...
April 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28365915/autoimmune-pancreatitis
#16
REVIEW
Shounak Majumder, Naoki Takahashi, Suresh T Chari
Autoimmune pancreatitis (AIP) is a chronic fibroinflammatory disease of the pancreas that belongs to the spectrum of immunoglobulin G-subclass4-related diseases (IgG4-RD) and typically presents with obstructive jaundice. Idiopathic duct-centric pancreatitis (IDCP) is a closely related but distinct disease that mimics AIP radiologically but manifests clinically most commonly as recurrent acute pancreatitis in young individuals with concurrent inflammatory bowel disease. IgG4 levels are often elevated in AIP and normal in IDCP...
April 1, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28351925/serum-igg2-and-tissue-igg2-plasma-cell-elevation-in-orbital-igg4-related-disease-igg4-rd-potential-use-in-igg4-rd-assessment
#17
Anita S Y Chan, Hardeep Mudhar, Sunny Yu Shen, Stephanie S Lang, Malee Fernando, Maryam Hazly Hilmy, Naomi Jayne Guppy, Ian Rennie, Lisa Dunkley, Issam Al Jajeh
AIMS: To determine the role of serum and tissue IgG2 in orbital biopsies with the histological features of IgG4-related disease (IgG4-RD) in comparison with non-IgG4-related orbital inflammatory disorders (OID), including autoimmune disorders. METHODS: This is an international (Sheffield, UK, and Singapore) collaborative, retrospective case review of 69 patients (38 from Singapore National Eye Centre and 31 from Royal Hallamshire Hospital, Sheffield) with orbital inflammatory biopsies between 2002 and 2016...
March 28, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28348556/t-cell-polarization-toward-th2-tfh2-and-th17-tfh17-in-patients-with-igg4-related-disease
#18
Aurélie Grados, Mikael Ebbo, Christelle Piperoglou, Matthieu Groh, Alexis Regent, Maxime Samson, Benjamin Terrier, Anderson Loundou, Nathalie Morel, Sylvain Audia, François Maurier, Julie Graveleau, Mohamed Hamidou, Amandine Forestier, Sylvain Palat, Emmanuelle Bernit, Bernard Bonotte, Catherine Farnarier, Jean-Robert Harlé, Nathalie Costedoat-Chalumeau, Frédéric Vély, Nicolas Schleinitz
IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder involving virtually every organ with a risk of organ dysfunction. Despite recent studies regarding B cell and T cell compartments, the disease's pathophysiology remains poorly understood. We examined and characterized subsets of circulating lymphocytes in untreated patients with active IgG4-RD. Twenty-eight consecutive patients with biopsy-proven IgG4-RD were included in a prospective, multicentric study. Lymphocytes' subsets were analyzed by flow cytometry, with analysis of TH1/TH2/TH17, TFH cells, and cytokine release by peripheral blood mononuclear cells...
2017: Frontiers in Immunology
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
#19
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
#20
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...
May 2017: Current Opinion in Rheumatology
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