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IgG4- related diseases

Kazunori Yamada, Masahiko Zuka, Kiyoaki Ito, Keishi Mizuguchi, Yasushi Kakuchi, Tamehito Onoe, Yasunori Suzuki, Masakazu Yamagishi, Shozo Izui, Marie Malissen, Bernard Malissen, Mitsuhiro Kawano
BACKGROUND: The adaptor protein Linker for activation of T cell (LAT) is a key signaling hub used by the T cell antigen receptor. Mutant mice expressing loss-of-function mutations affecting LAT and including a mutation in which tyrosine 136 is replaced by a phenylalanine (LatY136F) develop lymphoproliferative disorder involving T helper type 2 effector cells capable of triggering a massive polyclonal B cell activation that leads to hypergammaglobulinemia G1 and E and to non-resolving inflammation and autoimmunity...
2018: PloS One
Joo Hyung Lee, Hyung Sun Kim, Ji Sub Kim, Dong Ki Lee, Jin Hong Lim
RATIONALE: Immunoglobulin (Ig)G4-related pseudotumors of the liver are very rare diseases that are difficult to distinguish from malignant tumors. They can be usually improved by steroid therapy. Actinomycosis is a chronic, suppurative, granulomatous infection, for which immune suppression is a predisposing factor. It can also mimic malignant tumors. PATIENT CONCERNS: A 67-year-old man presented with mild abdominal discomfort and a 5-kg weight loss for 3 months...
June 2018: Medicine (Baltimore)
Liang-Jun Xie, Jian-Fang Li, Zhi Liu, Feng Zhang, Chang Zhao, Lu-Ping Qin, Ting-Jie Zhang, Mu-Hua Cheng
Immunoglobulin G4-related lung disease (IgG4-RLD) is a disease in which abundant activated IgG4-positive plasma cells and lymphocytes infiltrate lung tissues with high 18F-fluorodeoxyglucose uptake. Although various forms of radiologic features of IgG4-RLD have been reported, cavitating mass is a rare imaging feature and should be differentiated from cancer. Therefore, in this article, we report two cases both with unprovoked cough, bloody sputum and presenting quite similar cavitating lesions with high 18F-fluorodeoxyglucose uptake on positron emission tomography/ computed tomography, one of which diagnosed as IgG4-RLD and the other as lung cancer based on biopsy eventually...
December 2017: Archives of Rheumatology
Paul M Hoesly, Jason C Sluzevich
No abstract text is available yet for this article.
July 2018: JAAD Case Reports
J Brour, H Sassi, W Koubaa Mahjoub, M Ouederni, A Hassairi, A Chedly Debbiche, M Chéour
INTRODUCTION: Hyper-IgG4 syndrome is a rare cause of bilateral proptosis. It must always be considered after ruling out orbital lymphoma. CASE REPORT: We report a case of progressive bilateral proptosis for 4 years in a 34-year-old man. Orbital MRI showed an infiltrative process extending to the orbital fat, extraocular muscles and lacrimal glands. Lacrimal gland biopsy with immunohistochemical study showed a lymphoplasmocytic infiltrate rich in IgG4 and fibrosis...
June 7, 2018: Journal Français D'ophtalmologie
Youe Ree Kim, Young Hwan Lee, Keum-Ha Choi, Kwon-Ha Yoon
IgG4-related hepatopathy is usually referred to as parenchymal involvement of IgG4-related disease of the liver. As this disease entity was only recently described, little is known of its pathogenesis or imaging findings. We report a rare case of IgG4-related hepatopathy which presented as an ill-defined heterogeneously enhancing hepatic mass with diffusion restriction and a hepatobiliary phase defect in MR and CT images. Pathologic characteristics of the disease and its imaging findings are reviewed.
May 28, 2018: Clinical Imaging
Benjamin A Katchman, Meilin Zhu, Jennifer Blain Christen, Karen S Anderson
PURPOSE: Sweat is a relatively unexplored biofluid for diagnosis and monitoring of disease states. We present a proteomic study profiling immune-related biomarkers in sweat from healthy individuals. EXPERIMENTAL DESIGN: Eccrine sweat samples were collected from 50 healthy individuals. LC-MS/MS was performed on two pools of sweat samples from five male and female participants. Individual sweat samples were analyzed by antibody isotyping microarrays (n = 49), human cytokine arrays (n = 30), and quantitative ELISAs for interleukin-1α (n = 16), epidermal growth factor (n = 6), and angiogenin (n = 7)...
June 8, 2018: Proteomics. Clinical Applications
Alaa A Al Zaki, Shawna L Mann, Mollie N Carruthers, Graham W Slack, Luke Y C Chen
Lymphangiomas are benign tumors of the lymphatic vessels, which can be inflammatory and occasionally steroid-responsive. IgG4-related disease (IgG4-RD) is a recently defined fibro-inflammatory condition. We describe a novel association between reactive IgG4+ plasma cells and cystic lymphangioma in a young woman who had a dramatic clinical response to steroids.
June 2018: Clinical Case Reports
Mamta N Joshi, Benjamin Whitelaw, Paul V Carroll
Hypophysitis is a rare condition characterised by inflammation of the pituitary gland, usually resulting in hypopituitarism and pituitary enlargement. Pituitary inflammation can occur as a primary hypophysitis (most commonly lymphocytic, granulomatous or xanthomatous disease) or as secondary hypophysitis (as a result of systemic diseases, immunotherapy or alternative sella based pathologies). Hypophysitis can be classified using anatomical, histopathological and aetiological criteria. Non-invasive diagnosis of hypophysitis remains elusive, and the use of currently available serum anti-pituitary antibodies are limited by low sensitivity and specificity...
June 7, 2018: European Journal of Endocrinology
Ifeyinwa Emmanuela Obiorah, Alicia Henao Velasquez, Metin Özdemirli
No abstract text is available yet for this article.
June 5, 2018: Balkan Medical Journal
J Flores Balverdi, D F Baenas, N C Riscanevo, A V Sánchez, R Figueroa Rosales, A Alvarellos
CASE REPORT: The case is presented of a 64-year-old woman with bilateral palpebral swelling and dacryoadenitis, exophthalmos, and a history of chronic rhinitis and asthma. An increase in serum IgG4 was observed, and an incisional biopsy of lacrimal glands was performed, which showed fibrosis and a lymphoplasmacytic infiltrate with IgG4 producing cells. DISCUSSION: Orbital involvement in IgG4-related disease is frequent. Bilateral dacryoadenitis is the most common manifestation...
June 1, 2018: Archivos de la Sociedad Española de Oftalmología
Mitsuhiro Akiyama, Hidekata Yasuoka, Keiko Yoshimoto, Tsutomu Takeuchi
IgG4-related disease (IgG4-RD) is a systemic disorder characterized by elevated serum IgG4 level, which is mediated by T follicular helper 2 (Tfh2) cell. However, the cytokines responsible for enhancing IgG4 production remain unclear in IgG4-RD. The aim of this study was to identify responsible Tfh2-related cytokines (interleukin (IL)-4 and IL-21) for enhancing IgG4 production in IgG4-RD. Peripheral blood mononuclear cells obtained from consecutive patients with active, untreated IgG4-RD and healthy controls were examined...
May 31, 2018: Cytokine
Cory A Perugino, Sultan B AlSalem, Hamid Mattoo, Emanuel Della-Torre, Vinay Mahajan, Gayathri Ganesh, Hugues Allard-Chamard, Zachary Wallace, Sydney B Montesi, Johannes Kreuzer, Wilhelm Haas, John H Stone, Shiv Pillai
BACKGROUND: The antigenic trigger that drives the expansion of circulating plasmablasts and CD4+ cytotoxic T cells (CD4+ CTLs) in patients with IgG4-Related Disease (IgG4-RD) is presently unknown. OBJECTIVE: We sought to sequence Ig genes from single cell clones of dominantly-expanded plasmablasts and generate recombinant human monoclonal antibodies to identify relevant antigens in IgG4-RD using mass spectrometry. METHODS: Paired heavy and light chain cDNAs from dominant plasmablast clones were expressed as monoclonal antibodies (mAbs) and used to purify antigens using immunoaffinity chromatography...
May 28, 2018: Journal of Allergy and Clinical Immunology
Satoshi Takanashi, Mitsuhiro Akiyama, Katsuya Suzuki, Kotaro Otomo, Tsutomu Takeuchi
RATIONALE: Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disease characterized by elevated serum IgG4 levels with infiltration of IgG4+ plasma cells and severe fibrosis in affected tissues. Recently, idiopathic fibrosing mediastinitis (FM), an extremely rare fibroinflammatory disorder, has been recognized as a form of IgG4-RD. As IgG4-RD can be treated by glucocorticoids, identification of the etiology of FM by surgical biopsy is essential; however, mediastinal biopsy is often difficult...
June 2018: Medicine (Baltimore)
C A Simões, M R Tavares, N M M Andrade, T M Uehara, R A Dedivitis, C R Cernea
Riedel's thyroiditis (RT) represents one type of IgG4-related thyroid disease (IgG4RTD) and the diagnosis involves quantitative immunohistochemistry showing dense lymphoplasmacellular inflammatory infiltrate consisting of IgG4-positive plasma cells with storiform fibrosis and obliterative phlebitis. We report a case of RT with progressive enlargement of the anterior neck, severe dysphagia, odynophagia, and dyspnea. The patient underwent surgical decompression of the airway, protection tracheotomy, and gastrostomy for nutritional intake 6 months after first symptoms...
2018: Case Reports in Endocrinology
Warren Fong, Ian Liew, Damien Tan, Kiat Hon Lim, Albert Low, Ying Ying Leung
OBJECTIVES: To describe the features and treatment outcomes of IgG4-RD in multi-ethnic patients in Singapore. METHODS: Retrospective study was performed on IgG4-RD patients identified from patient databases in a tertiary hospital. RESULTS: Fourty-two patients (76% male) were included; 79% fulfilled the 2011 comprehensive diagnostic criteria for IgG4-RD for definite IgG4-RD. 81% were Chinese and 19% were Malays. Common initial manifestations included jaundice (52%), abdominal pain (36%) and swollen salivary glands (26%)...
May 24, 2018: Clinical and Experimental Rheumatology
Takanori Sasaki, Mitsuhiro Akiyama, Yuko Kaneko, Hidekata Yasuoka, Katsuya Suzuki, Kunihiro Yamaoka, Tsutomu Takeuchi
OBJECTIVES: To identify risk factors of relapse in IgG4-related disease (IgG4-RD) during glucocorticoid (GC) tapering. METHODS: A total of 27 consecutive patients with IgG4-RD (7 with and 20 without relapse) treated with GC for more than 6 months were enrolled. Baseline characteristics were compared in patients with and without relapse. Longitudinal analysis was also performed. RESULTS: Patients with relapse had significantly higher levels of serum IgG4 (816...
May 24, 2018: Clinical and Experimental Rheumatology
Mohammad Almeqdadi, Mohammed Al-Dulaimi, Aleksandr Perepletchikov, Kevin Tomera, Bertrand L Jaber
Retroperitoneal fibrosis (RPF) is a progressive fibroinflammatory disease that can be complicated by urinary obstruction. RPF can be the only manifestation of IgG4-related disease (IgG4-RD). Treatment of IgG4-related RPF is challenging and mostly consists of long-term glucocorticoids leading to significant side effects and treatment intolerance. Recent exploration of the role of rituximab as a B-cell depleting therapy in the treatment of IgG4-RD provides therapeutic potential as a well-tolerated alternative to glucocorticoids...
2018: Clinical Nephrology. Case Studies
S Azuma, A Kurita, S Yazumi
No abstract text is available yet for this article.
May 28, 2018: Journal of Gastroenterology and Hepatology
Ilaria Puxeddu, Riccardo Capecchi, Filippo Carta, Antonio Gaetano Tavoni, Paola Migliorini, Roberto Puxeddu
IgG4-related disease (IgG4-RD) is a rare fibroinflammatory condition that can affect almost any organ, characterized by swollen lesions and often by eosinophilia and elevated serum IgG4 concentrations. The diagnosis of IgG4-RD is a challenging task: in fact, single or multiple organs can be affected and clinical, serological, and histological findings can be heterogeneous. In IgG4-RD, the involvement of salivary glands is observed in 27% to 53% of patients. Several organ-specific conditions, now recognized as different manifestations of IgG4-related sialadenitis (IgG4-RS), were viewed in the past as individual disease entities...
2018: Journal of Immunology Research
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