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IgG4- related diseases

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https://www.readbyqxmd.com/read/28107092/a-solid-pseudopapillary-neoplasm-of-the-pancreas-associated-with-igg4-related-pancreatitis
#1
Yuichiro Hatano, Keisuke Kawashima, Takuji Iwashita, Masaki Kimura, Masahito Shimizu, Akira Hara
A solid and cystic pancreatic lesion was incidentally found in a 62-year-old woman on abdominal computed tomography. The lesion was diagnosed as a solid pseudopapillary neoplasm by using endoscopic ultrasound-guided fine needle aspiration, and the tumor was resected. Histologically, the tumor cells had relatively small, round nuclei and papillary-like architecture in a hemorrhagic background. On immunohistochemical examination, the tumor cells were diffusely positive for nuclear β-catenin and cytoplasmic CD10...
November 1, 2016: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28105551/elevated-serum-igg4-levels-in-diagnosis-and-treatment-response-in-patients-with-idiopathic-retroperitoneal-fibrosis
#2
L G Pelkmans, T R Hendriksz, P J Westenend, H J Vermeer, E F H van Bommel
Idiopathic retroperitoneal fibrosis (iRPF) may be a manifestation of IgG4-related disease. Measuring serum IgG4 (sIgG4) may be of value in monitoring iRPF, but this has scarcely been evaluated. It is unknown if tamoxifen (TMX) affects sIgG4 levels. We performed a prospective inception cohort study of 59 patients with untreated (re)active iRPF stratified by elevated (>1.4 g/L) or normal sIgG4 level. Changes in sIgG4 levels following TMX initiation and, if treatment failed, during subsequent corticosteroid (CS) treatment were analyzed...
January 19, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28101487/immunoglobulin-g4-related-thyroid-diseases
#3
REVIEW
Dulani Kottahachchi, Duncan J Topliss
Immunoglobulin G4-related disease (IgG4-RD) is a new disease category involving many organ systems, including the endocrine system in general and the thyroid in particular. Since an initial association was made between hypothyroidism and autoimmune (IgG4-related) pancreatitis, more forms of IgG4-related thyroid disease (IgG4-RTD) have been recognized. Four subcategories of IgG4-RTD have so far been identified: Riedel thyroiditis (RT), fibrosing variant of Hashimoto thyroiditis (FVHT), IgG4-related Hashimoto thyroiditis, and Graves disease with elevated IgG4 levels...
December 2016: European Thyroid Journal
https://www.readbyqxmd.com/read/28091934/roles-of-plasmablasts-and-b-cells-in-igg4-related-disease-implications-for-therapy-and-early-treatment-outcomes
#4
Marco Lanzillotta, Emanuel Della-Torre, John H Stone
High serum IgG4 concentrations are a striking feature of many patients with IgG4-related disease (IgG4-RD). Blood levels of IgG4 often reach ten, twenty, and even thirty or more times higher than the upper limit of normal. Under the proper clinical circumstances, the finding of an elevated serum IgG4 concentration serves as a useful biomarker for the diagnosis of this condition. This serum IgG4 elevation quickly called attention to the possibility of therapies targeting cells of the B lymphocyte lineage. In addition, a greater understanding of the cellular mechanisms that underpin IgG4-RD has identified peripheral blood plasmablasts as a promising biomarker for this disease...
January 7, 2017: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/28091932/current-concept-of-igg4-related-disease
#5
Kazuichi Okazaki, Hisanori Umehara
IgG4-related disease (IgG4-RD) is a fibroinflammatory disease of unknown etiology, which is characterized by a tendency to form tumefactive lesions, increased serum levels of IgG4, and massive infiltration of IgG4-positive plasma cells with storiform fibrosis and/or obliterative phlebitis. Patients with IgG4-RD have frequently multiorgan involvements such as the pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, and retroperitoneum. IgG4-RD mainly affects middle-aged to elderly men except for involvement in lachrymal and salivary glands, so-called Mikulicz's disease...
December 28, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/28079853/solely-lung-involved-igg4-related-disease-a-case-report-and-review-of-the-literature
#6
Xiao-Qin Zhang, Guo-Ping Chen, Sheng-Chang Wu, Sa Yu, Hong Wang, Xuan-Yi Chen, Zhuo-Chao Ren
By analyzing the clinical data of 1 case of IgG4-related lung disease(IgG4-RLD) and the review of literature, the author investigated the clinical characteristics of IgG4-RLD. IgG4-RLD is a rare disease characterized by significant elevation of serum IgG4 and infiltration of a large number of IgG4+ plasma cells. The clinical manifestations of the disease were nonspecific, and the imaging features were mixed with several types. The disease can only be involved in the lung, but also multiple organ involvement...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28078088/immunoglobulin-g4-related-disease-presenting-as-a-pulmonary-nodule-with-an-irregular-margin
#7
Tetsuyuki Okubo, Yumiko Oyamada, Masaya Kawada, Yo Kawarada, Shuji Kitashiro, Shunichi Okushiba
We report a case of immunoglobulin G4 (IgG4)-related lung disease presenting as a solitary pulmonary nodule with an irregular margin on computed tomography. The nodule showed a high standardized uptake value on positron emission tomography. A malignant pulmonary tumour could not be excluded. Middle lobectomy was performed. Histological analysis revealed marked lymphoplasmacytic infiltration and storiform fibrosis. Immunostaining indicated the presence of IgG4-positive plasma cells. A definitive diagnosis of IgG4-related disease was confirmed...
January 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28072985/-recent-advance-of-igg4-related-thyroid-disease
#8
S Wang, R E Feng
No abstract text is available yet for this article.
January 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28070877/idiopathic-retroperitoneal-fibrosis-and-its-overlap-with-igg4-related-disease
#9
REVIEW
Giovanni Maria Rossi, Rossana Rocco, Eugenia Accorsi Buttini, Chiara Marvisi, Augusto Vaglio
Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis...
January 9, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28060780/18f-fdg-pet-ct-features-of-chronic-sclerosing-sialadenitis-presenting-as-localized-igg4-related-disease
#10
Nobuo Kashiwagi, Eisuke Enoki, Chisa Hosokawa, Kenta Sakaguchi, Takamichi Murakami
Chronic sclerosing sialadenitis is a benign inflammatory condition that most commonly affects the submandibular gland in elderly individuals. It is currently known to belong to the spectrum of IgG4-related systemic diseases, which is reflected by systemic involvement on F-FDG PET/CT images. We presented a case of a 73-year-old man with histologically proven IgG4-related chronic sclerosing sialadenitis, unilateral localized form, on whole-body F-FDG PET/CT images that mimicked submandibular carcinoma with lymph node metastasis...
February 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28055067/igg4-related-disease-with-orbital-pseudotumors-treated-with-rituximab-combined-with-palpebral-surgery
#11
Iman Aouidad, Pierre Schneider, Mathieu Zmuda, Jeremy Gottlieb, Manuelle Viguier
No abstract text is available yet for this article.
January 4, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28049999/overlap-of-post-obstructive-diuresis-and-unmasked-diabetes-insipidus-in-a-case-of-igg4-related-retroperitoneal-fibrosis-and-tuberoinfundibular-hypophysitis-a-case-report-and-review-of-the-literature
#12
Midori Sasaki Yatabe, Kimio Watanabe, Yoshimitsu Hayashi, Junichi Yatabe, Satoshi Morimoto, Atsuhiro Ichihara, Masaaki Nakayama, Tsuyoshi Watanabe
The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28039416/symptomatic-unilateral-sacroiliitis-as-a-first-presenting-feature-of-igg4-related-disease-with-successful-response-to-treatment-after-1-year-of-follow-uparticle
#13
Anna Jamnitski, Odile van Hall, Niek de Vries, Paul van der Valk, Willem F Lems, Irene E van der Horst-Bruinsma
No abstract text is available yet for this article.
December 30, 2016: Rheumatology
https://www.readbyqxmd.com/read/28036157/rituximab-hypersensitivity-in-igg4-related-disease-successful-desensitization-in-a-patient-with-igg4-rheumatoid-factor
#14
Emanuel Della-Torre, Antonio Conti, Alvise Berti, Mona Rita Yacoub, Massimo Alessio
No abstract text is available yet for this article.
December 30, 2016: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28035701/three-unusual-histopathological-presentations-of-angiolymphoid-hyperplasia-with-eosinophilia
#15
Angel Fernandez-Flores, David S Cassarino
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a poorly understood benign vasculoproliferative disorder. Histopathologicaly, the lesions are composed of a dermal or subcutaneous proliferation of blood vessels of varying sizes with plump and bland endothelial cells, which often show vacuolization and protrusion of the cytoplasm into the lumen. The vascular proliferation is admixed with a diffuse inflammatory infiltrate composed of lymphocytes, eosinophils, and mast cells. Lymphoid follicles with germinal centers can sometimes be seen...
December 30, 2016: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28029020/clinical-update-in-aspects-of-the-management-of-autoimmune-thyroid-diseases
#16
REVIEW
Duncan J Topliss
Aspects of autoimmune thyroid disease updated in this review include: immunoglobulin G4 (IgG4)-related thyroid disease (Riedel's thyroiditis, fibrosing variant of Hashimoto's thyroiditis, IgG4-related Hashimoto's thyroiditis, and Graves' disease with elevated IgG4 levels); recent epidemiological studies from China and Denmark indicating that excess iodine increases the incidence of Hashimoto's thyroiditis and hypothyroidism; immunomodulatory agents (ipilimumab, pembrolizumab, nivolumab) activate immune response by inhibiting T-cell surface receptors which down-regulate immune response, i...
December 2016: Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28027743/igg4-related-cardiovascular-disease-the-emerging-role-of-cardiovascular-imaging
#17
REVIEW
Sophie Mavrogeni, George Markousis-Mavrogenis, Genovefa Kolovou
Immunoglobulin 4-related disease (IgG4-related disease) is a systemic inflammatory disease that presents with increases of serum IgG4. It may affect various systems, including the cardiovascular (CV) system. Assessment of serum IgG4 levels and involved organ biopsy are necessary for diagnosis. IgG4-related disease is characterized by fibrosclerosis, lymphocytic infiltration and presence of IgG4-positive plasma cells. The disease usually responds to treatment with corticosteroids and/or immunosuppressive medication...
January 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28025265/affection-of-the-cardiovascular-system-by-igg4-related-disease
#18
Tareq Ibrahim, Daniela Muenzel, Gregor Babaryka, Petra Barthel, Klaus Thürmel
No abstract text is available yet for this article.
December 26, 2016: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28012876/in-search-of-a-disease
#19
Gustavo Savino, Andrea Campana, Gianluigi Petrone, Gabriela Grimaldi, Ann P Murchison
A 10-year-old child presented with right eyelid swelling and a palpable orbital mass. Diagnostic evaluation with the aid of excisional biopsy revealed a pseudo- encapsulated mass associated with high levels of serum perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA). Family history of rheumatic diseases, response to treatment with corticosteroids and immunosuppressant drugs and clinical and histopathological features suggested an orbital autoimmune response in which p-ANCA antibodies were detected, and a diagnosis of probable IgG4-related disease was made...
December 21, 2016: Survey of Ophthalmology
https://www.readbyqxmd.com/read/28005278/igg4-related-disease-and-lymphocyte-variant-hypereosinophilic-syndrome-a-comparative-case-series
#20
Mollie N Carruthers, Sujin Park, Graham W Slack, Bakul I Dalal, Brian F Skinnider, David F Schaeffer, Jan P Dutz, Joanna K Law, Fergal Donnellan, Vladimir Marquez, Michael Seidman, Patrick C Wong, Andre Mattman, Luke Y C Chen
OBJECTIVE: To compare the clinical and laboratory features of IgG4-related disease (IgG4-RD) and lymphocyte variant-hypereosinophilic syndrome (L-HES), two rare diseases that often present with lymphadenopathy, gastrointestinal symptoms, eosinophilia and elevated immunoglobulins/IgE. METHOD: Comparative case series of 31 IgG4-RD and 13 L-HES patients. RESULTS: Peripheral blood eosinophilia was present in 8/31 IgG4-RD patients compared to 13/13 L-HES patients (median eosinophils 0...
December 22, 2016: European Journal of Haematology
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