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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/27891673/igg4-related-disease-experience-of-100-consecutive-cases-from-a-specialist-centre
#1
Adrian C Bateman, Emma L Culver
AIMS: To describe the features of 100 consecutive cases referred to a single UK institution, in which a diagnosis of IgG4-related disease (IgG4-RD) was under consideration. METHODS AND RESULTS: The histological features were reviewed by a single histopathologist and cases categorised using the 2012 Boston criteria: Category 1 - histologically highly suggestive of IgG4-RD; 2 - probable histopathological features of IgG4-RD; 3 - insufficient histopathological features of IgG4-RD...
November 28, 2016: Histopathology
https://www.readbyqxmd.com/read/27846767/a-multicenter-phase-ii-prospective-clinical-trial-of-glucocorticoid-for-patients-with-untreated-igg4-related-disease
#2
Yasufumi Masaki, Shoko Matsui, Takako Saeki, Hiroto Tsuboi, Shintaro Hirata, Yasumori Izumi, Taiichiro Miyashita, Keita Fujikawa, Hiroaki Dobashi, Kentaro Susaki, Hisanori Morimoto, Kazutaka Takagi, Mitsuhiro Kawano, Tomoki Origuchi, Yoko Wada, Naoki Takahashi, Masanobu Horikoshi, Hiroshi Ogishima, Yasunori Suzuki, Takafumi Kawanami, Haruka Iwao Kawanami, Tomoyuki Sakai, Yoshimasa Fujita, Toshihiro Fukushima, Masatoshi Saito, Ritsuro Suzuki, Yuko Morikawa, Tadashi Yoshino, Shigeo Nakamura, Masaru Kojima, Nozomu Kurose, Yasuharu Sato, Yoshiya Tanaka, Susumu Sugai, Takayuki Sumida
"Objective" Although glucocorticoids are effective for patients with IgG4-related disease, the treatment has not yet been standardized. Therefore, the treatment strategy should be established. "Patients and methods" Patients who fulfilled the comprehensive diagnostic criteria for definite IgG4-related disease were started on prednisolone (0.6mg/kg body weight) with the dose reduced every 2 weeks. The subsequent maintenance dose and need for prednisolone were determined for individual patients. The primary endpoint was the complete remission (CR) rate at 1 year...
November 16, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27831514/diagnostic-challenges-in-a-case-of-igg4-rd-affecting-the-temporal-bone
#3
Jackson Ross Vuncannon, Nicholas John Panella, Kelly R Magliocca, Douglas E Mattox
INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is a recently described fibroinflammatory condition with a characteristic histology. While IgG4-RD can affect a great variety of anatomical sites, it has been seldom described in the temporal bone. METHODS: Herein, a case IgG4-RD occurring in the temporal bone of a 35-year-old woman is reported. DISCUSSION: This case of IgG4-RD of the temporal bone proved a uniquely challenging diagnosis due to slightly atypical histology falling outside of "highly suggestive" criteria...
November 9, 2016: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/27817178/roles-of-regulatory-t-and-b-cells-in-igg4-related-disease
#4
Kazushige Uchida, Kazuichi Okazaki
Immunoglobulin G4 (IgG4) -related disease (RD) is a newly recognized systemic disease. Although there are several forms of IgG4-RD reported under various names, depending on the target organ and characteristics, patients with IgG4-RD manifest several immunologic and histologic abnormalities including increased levels of serum IgG4 and storiform fibrosis with infiltration of lymphocytes and IgG4-positive plasmacytes in the involved organs. However, the pathophysiology remains unclear. Regulatory immune cells play an important role in several immune-related diseases...
November 6, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27801343/-a-retrospective-case-control-study-of-immunoglobulin-g4-related-disease-combined-with-malignancy
#5
Y L Feng, D Wu, S Y Zhang, X Wu, H J Shu, A M Yang, J M Qian
IgG4-related disease (IgG4-RD) has been proved to be associated with malignancy.The incidence and risk factors of malignancy development in IgG4-RD were not clear. Nine IgG4-RD patients with malignancies and 27 IgG4-RD control cases were analyzed for risk factors and clinical features. The incidence of malignancy in IgG4-RD was 3.3%, higher than age-controlled general population. Smoking history was significantly more common in patients with malignancies than in the control group (9/9 vs 16/27, P<0.05). A total of 6/9 malignancies occurred within the first year after the diagnosis of IgG4-RD...
November 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27799828/serum-total-igg-and-igg4-levels-in-thyroid-eye-disease
#6
Aileen Sy, Rona Z Silkiss
PURPOSE: To investigate the relationship between immunoglobulin G (IgG)4-related disease (IgG4-RD) and thyroid eye disease (TED) with respect to IgG levels. PATIENTS AND METHODS: A retrospective review of total IgG, IgG subclass, and thyroid stimulating immunoglobulin (TSI) levels in 24 patients with TED. RESULTS: Five patients (20.8%) demonstrated serum IgG4 levels consistent with IgG4-RD without any additional systemic disease. Total IgG and IgG subclass levels were found to be an inadequate proxy for TSI elevation...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27797069/igg4-related-sclerosing-mesenteritis-causing-bowel-obstruction-a-case-report
#7
Atsushi Abe, Tatsuya Manabe, Nobuyoshi Takizawa, Takashi Ueki, Daisuke Yamada, Kinuko Nagayoshi, Yoshihiko Sadakari, Hayato Fujita, Shuntaro Nagai, Hidetaka Yamamoto, Yoshinao Oda, Masafumi Nakamura
Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease primarily involving the small-bowel mesentery. Recently, SM was reported to be closely related to IgG4-related disease (IgG4-RD). This report describes a patient with SM associated with IgG4-RD. A 77-year-old woman with a history of surgery for ectopic pregnancy and wound dehiscence presented with intestinal obstruction. Abdominal enhanced computed tomography (CT) revealed an enhanced, radially shaped, oval mass, 3 cm in diameter, with an unclear rim in the mesentery of the distal ileum, which may have involved the distal ileum...
December 2016: Surgical Case Reports
https://www.readbyqxmd.com/read/27785920/a-case-of-igg4-related-tubulointerstitial-nephritis-and-membranous-glomerulonephritis-during-the-clinical-course-of-gastric-cancer-imaging-features-of-igg4-related-kidney-disease
#8
Shigeto Horita, Hiroshi Fujii, Ichiro Mizushima, Yuhei Fujisawa, Satoshi Hara, Kazunori Yamada, Dai Inoue, Kenichi Nakajima, Kenichi Harada, Mitsuhiro Kawano
We describe an 81-year-old man with immunoglobulin G4-related disease (IgG4-RD) presenting with submandibular gland, lymph node, lung, kidney, aortic wall, and prostate lesions with concomitant gastric cancer. After curative surgical treatment of the gastric cancer, corticosteroid therapy for progressively decreasing renal function was started. Before starting steroid therapy, fluorodeoxyglucose positron emission tomography-computed tomography revealed multiple lesions of IgG4-RD but no metastasis of the cancer...
October 27, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27766198/igg4-related-lung-disease-extending-to-the-thoracic-vertebrae
#9
Alexandros Stamatopoulos, Davide Patrini, Efstratios Koletsis, Elaine Borg, Reena Khiroya, Martin Hayward, David Lawrence, Nikolaos Panagiotopoulos
IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect practically every organ. Although it was first identified in pancreas and salivary glands, major organs like liver, biliary tree, kidney, thyroid glands and lungs are commonly involved, sometimes resulting in organ failure. We describe a case of an 41-year-old man presented with back pain after a rotator cuff injury. A Computed Tomography (CT) revealed incidentally a right lower lobe paravertebral lesion extending across the T5 and T6 vertebral levels and invading into the adjacent pleural surface...
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27744510/th1-th2-immune-balance-and-other-t-helper-subsets-in-igg4-related-disease
#10
Masafumi Moriyama, Seiji Nakamura
IgG4-related disease (IgG4-RD) is a systemic disease characterized by elevated serum IgG4 levels and a strong infiltration of IgG4-positive plasma cells in various organs. IgG4-RD patients also frequently suffer from allergic diseases, including asthma and atopic dermatitis. It is well known that T helper type 2 (Th2) cells have an important role in the initiation of allergic diseases, and Th2 cytokines such as interleukin (IL)-4 and IL-13 promote class switching to IgG4. Therefore, IgG4-RD is considered to be a Th2-predominant disease...
October 16, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27744509/igg4-related-disease-and-innate-immunity
#11
Tomohiro Watanabe, Kouhei Yamashita, Masatoshi Kudo
An increased number of clinicopathological studies on autoimmune pancreatitis, cholangitis, and sialoadenitis have led to the recognition of immunoglobulin G4-related disease (IgG4-RD) as a novel disorder, characterized by elevated levels of serum IgG4 and infiltration of IgG4-expressing plasma cells in the affected organs. Although the immunological background associated with the development of IgG4-RD remains poorly understood, recent studies have suggested involvement of the innate immune response in its pathogenesis...
October 16, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27729479/igg4-related-disease-of-bilateral-temporal-bones
#12
Lilun Li, Bryan Ward, Margaret Cocks, Amir Kheradmand, Howard W Francis
OBJECTIVE: IgG4-related disease (IgG4-RD) is an idiopathic inflammatory condition that causes pseudotumor formation in single or multiple organs, including those of the head and neck. Temporal bone involvement is rare, with only 3 cases of unilateral temporal bone IgG4-RD described in the literature. We report the first known case of IgG4-RD of bilateral temporal bones and describe its clinical presentation, diagnosis, and treatment. METHODS: The patient was a 52-year-old man with latent tuberculosis (TB) who presented with a 10-year history of bilateral profound hearing loss and vestibular dysfunction...
October 10, 2016: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/27726003/the-immunobiology-of-immunoglobulin-g4-and-complement-activation-pathways-in-igg4-related-disease
#13
Shigeyuki Kawa
High serum immunoglobulin (Ig) G4 concentration and abundant IgG4-bearing plasma cell infiltration are characteristic features in autoimmune pancreatitis (AIP). AIP is also complicated with a variety of other organ involvements that commonly share marked IgG4-bearing plasma cell infiltration, suggesting the existence of a systemic disease associated with IgG4 currently recognized as IgG4-related disease (IgG4-RD). However, it is controversial whether IgG4 plays a role in the pathogenesis of AIP or IgG4-RD through such characteristic attributes as Fab-arm exchange and rheumatoid factor (RF)-like activity...
September 21, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/27720730/no-definite-clinical-features-of-igg4-related-disease-in-patients-with-pulmonary-nodular-lymphoid-hyperplasia
#14
Melanie C Bois, Hiroshi Sekiguchi, Jay H Ryu, Eunhee S Yi
Pulmonary nodular lymphoid hyperplasia (PNLH) may show heavy lymphoplasmacytic infiltrates with increased Immunoglobulin G4 (IgG4)-positive plasma cells as in IgG4-related disease (IgG4-RD). However, it is unclear whether PNLH could be a manifestation of IgG4-RD. A recent study reported the presence of Epstein-Barr virus (EBV) in IgG4-related lymphadenopathy. We postulated that a subset of PNLH might represent IgG4-related lung disease (IgG4-RLD) with EBV-positive lymphocytes as has been reported in IgG4-related lymphadenopathy...
October 5, 2016: Human Pathology
https://www.readbyqxmd.com/read/27686866/ocular-adnexal-marginal-zone-lymphoma-arising-in-a-patient-with-igg4-related-ophthalmic-disease
#15
Kenji Nishida, Yuka Sogabe, Ayako Makihara, Akemi Senoo, Hisanori Morimoto, Mai Takeuchi, Yuka Gion, Tadashi Yoshino, Yasuharu Sato
A 41-year-old man was diagnosed with immunoglobulin G4-related disease (IgG4-RD) in both eyelids 4 years ago and exhibited good response to steroid therapy. However, rapid swelling of the right eyelid lesion was recently observed. As IgG4-RD progression was suspected, biopsy was performed. Although the histology was consistent with IgG4-RD, the infiltrating large atypical lymphoid cells showed immunoglobulin light-chain restriction and IgH gene rearrangement. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells...
August 11, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27667138/-how-i-manage-igg4-related-disease
#16
Emanuel Della-Torre, John H Stone
IgG4-related disease (IgG4-RD) is an immune-mediated disease driven by interactions on several levels between cells of the B and T lineages. A key driver of IgG4-RD is believed to be a novel CD4+ cytotoxic T lymphocyte that bears SLAM-F7 on its surface. This cell is presumed to be sustained by continuous antigen presentation by cells of the B cell lineage, particularly plasmablasts. Because IgG4-RD has been recognized for fewer than 15 years as a unified diagnostic entity, no controlled clinical trials or few prospective studies of any sort have been performed...
November 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27666423/fine-needle-aspiration-cytology-of-igg4-related-disease-a-potential-diagnostic-pitfall
#17
Rajwant Kaur, Suvradeep Mitra, Arvind Rajwanshi, Ashim Das, Uma Nahar Saikia, Pranab Dey
BACKGROUND: IgG4-related disease (IgG4-RD) is a tumefactive fibro-inflammatory lesion that can affect any organ system in the body. Till date, no cytological data on IgG4-RD are available and this is the first study depicting the cytopathology features of IgG4-RD. AIM AND OBJECTIVE: To describe the cytopathological features and potential diagnostic errors of IgG4-RD. MATERIALS AND METHODS: The cytological features of 10 histopathology proven IgG4-RD cases (11 samples) were retrospectively reviewed along with corresponding histopathology...
September 26, 2016: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/27647429/riedel-s-thyroiditis-association-with-igg4-related-disease
#18
Marius N Stan, Vikram Sonawane, Thomas J Sebo, Prabin Thapa, Rebecca S Bahn
CONTEXT: IgG4-positive (+) plasma cells have been reported in both Riedel's thyroiditis (RT) and Hashimoto's thyroiditis (HT). These cells are the hallmark of IgG4-related disease (IgG4-RD). OBJECTIVE: We sought to determine whether RT is part of IgG4-RD spectrum. DESIGN, SETTING AND PATIENTS: This was a case-control study performed at a tertiary medical centre. We included RT cases from the period 1958 to 2008 that had sufficient paraffin-embedded tissue for IgG4 immunostaining...
September 20, 2016: Clinical Endocrinology
https://www.readbyqxmd.com/read/27622319/deconstructing-igg4-related-disease-involvement-of-midline-structures-comparison-to-common-mimickers
#19
Marco Lanzillotta, Corrado Campochiaro, Matteo Trimarchi, Gianluigi Arrigoni, Simonetta Gerevini, Raffaella Milani, Enrica Bozzolo, Matteo Biafora, Elena Venturini, Maria Pia Cicalese, John H Stone, Maria Grazia Sabbadini, Emanuel Della-Torre
OBJECTIVE: A series of destructive and tumefactive lesions of the midline structures have been recently added to the spectrum of IgG4-related disease (IgG4-RD). We examined the clinical, serological, endoscopic, radiological, and histological features that might be of utility in distinguishing IgG4-RD from other forms of inflammatory conditions with the potential to involve the sinonasal area and the oral cavity. METHODS: We studied 11 consecutive patients with erosive and/or tumefactive lesions of the midline structures referred to our tertiary care center...
September 13, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27609186/igg4-related-disease-causing-facial-nerve-and-optic-nerve-palsies-case-report-and-literature-review
#20
Cameron C Wick, Joseph Zachariah, Sunil Manjila, William C Brown, Prerna Malla, Bashar Katirji, Mark Cohen, Cliff A Megerian
IgG4-related disease (IgG4-RD) is increasingly being recognized as an entity effecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also involve the temporal bone and skull base. We report a rare care of a 61-year-old female with IgG4-RD presenting as synchronous lesions of the middle ear and middle cranial fossa with polyneuropathy of cranial nerves II, VI, and VII. Initial histopathological evaluation of her resected ear mass suggested a benign inflammatory process but no specific diagnosis...
August 18, 2016: American Journal of Otolaryngology
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