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IgG4-related disease

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https://www.readbyqxmd.com/read/28346259/igg4-related-ophthalmic-disease-presenting-as-choroidal-and-orbital-lesions
#1
William S Gange, Stephen M Holland, Felipe De Alba
PURPOSE: To report a case of IgG4-related ophthalmic disease (IgG4-ROD) which presented as choroidal and orbital lesions. METHODS: Case report. RESULTS: A 64-year-old man presented with left eye photopsias and a history of IgG4-related perirenal fibrosis. Fundoscopic examination showed multiple bilateral yellow choroidal lesions, and optical coherence tomography showed multiple choroidal lesions. Magnetic resonance imaging of the orbits showed an enhancing lesion present circumferential to the optic nerve, but greater medially, abutting the posterior surface of the left globe...
March 24, 2017: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/28331012/igg4-related-disease-a-new-entity-sometimes-needs-a-retrospective-diagnosis
#2
Maristela Böhlke, Gabriel Santos
No abstract text is available yet for this article.
April 2017: Postgraduate Medical Journal
https://www.readbyqxmd.com/read/28328835/poor-response-of-initial-steroid-therapy-for-igg4-related-sclerosing-cholangitis-with-multiple-organs-affected
#3
Wei Liu, Weijie Chen, Xiaodong He, Qiang Qu, Tao Hong, Binglu Li
IgG4-related sclerosing cholangitis (IgG4-SC) is a rare biliary manifestation in which many other organs might be affected. The purpose of our study was to investigate the different clinical characteristics and initial steroid response between IgG4-SC patients with and without other organs affected.A series of patients with IgG4-SC in the period from January 2006 to December 2015 at our hospital were included. The pancreas and major salivary glands were screened, and the initial corticosteroid therapy was given...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28322216/igg4%C3%A2-related-disease-as-an-unusual-cause-of-biliary-stenosis-and-lymphadenopathy
#4
Magdalena Stojek, Grzegorz Rymkiewicz, Marian Smoczyński, Wojciech Biernat, Krystian Adrych, Jan M Zaucha
No abstract text is available yet for this article.
March 21, 2017: Pol Arch Intern Med
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
#5
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/28321597/bilateral-renal-hilar-tumors-in-igg4-related-disease
#6
Yosuke Hirabayashi, Eiji Ishikawa, Masaaki Ito
No abstract text is available yet for this article.
March 21, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28319486/igg4-related-disease
#7
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
https://www.readbyqxmd.com/read/28314688/nivolumab-treatment-for-oesophageal-squamous-cell-carcinoma-an-open-label-multicentre-phase-2-trial
#8
Toshihiro Kudo, Yasuo Hamamoto, Ken Kato, Takashi Ura, Takashi Kojima, Takahiro Tsushima, Shuichi Hironaka, Hiroki Hara, Taroh Satoh, Satoru Iwasa, Kei Muro, Hirofumi Yasui, Keiko Minashi, Kensei Yamaguchi, Atsushi Ohtsu, Yuichiro Doki, Yuko Kitagawa
BACKGROUND: Nivolumab is a human monoclonal IgG4 antibody that inhibits programmed cell death protein 1 (PD-1) expressed on activated T cells. We investigated the safety and activity of nivolumab in patients with treatment-refractory oesophageal cancer. METHODS: We did an open-label, single-arm, multicentre phase 2 study. Eligible patients had advanced squamous-cell carcinoma, adenosquamous-cell carcinoma, or adenocarcinoma of the oesophagus refractory or intolerant to fluoropyrimidine-based, platinum-based, and taxane-based chemotherapy...
March 14, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28304239/immunoglobulin-g4-related-epidural-inflammatory-pseudotumor-presenting-with-pulmonary-complications-and-spinal-cord-compression-case-report
#9
Kavelin Rumalla, Kyle A Smith, Paul M Arnold
Immunoglobulin G4-related disease (IgG4-RD) is a recently defined condition characterized by inflammatory tumefactive lesions in various organ systems. IgG4-RD is a clinical and radiological diagnosis of exclusion and requires the presence of specific histopathological criteria for diagnosis. A 50-year-old man presented to an outside hospital with a 3-month history of progressively worsening back pain and symptoms of pleurisy, nasal crusting, and hematochezia. Radiological workup revealed an epidural-paraspinal mass with displacement of the spinal cord, destruction of the T5-6 vertebrae, and extension into the right lung...
March 17, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28299186/recent-advances-in-understanding-and-managing-igg4-related-disease
#10
REVIEW
Anna R Wolfson, Daniel L Hamilos
IgG4-related disease was only recently discovered, so its description, management, and new discoveries related to its etiology are rapidly evolving. Because IgG4 itself is a unique antibody which is intimately related to the diagnosis of the disease, the role of plasmablasts in the pathophysiology remains an active area of discussion. Recent studies have uncovered a possible role for CD4-positive cytotoxic T lymphocytes, T follicular helper cells, and M2 macrophages. The clinical presentation is variable and can be vague, as this disease affects many organs and new presentations are continuing to be described...
2017: F1000Research
https://www.readbyqxmd.com/read/28297814/-igg4-related-lung-disease-analysis-of-8-cases-and-literature-review
#11
G J Han, H Hu, D Mao, X Bai, D Y She, S F Zhao, Z L Wen, J Gao
Objective: To improve the understanding and treatment of IgG4-related lung disease (IgG4-RLD). Methods: The clinical characteristics, serum IgG4 levels, pathological features, chest CT, therapy and prognosis of 8 patients with IgG4-RLD were retrospectively analyzed. These patients were admitted to the People's Liberation Army General Hospital and the pathological diagnosis was made between December 2005 and March 2016. Relevant literatures were reviewed. Results: The 8 patients with IgG4-RLD included 4 men and 4 women, with an average age of (59±4) years (range, 37-74)...
March 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28297756/-igg4-immunohistochemistry-in-riedle-thyroiditis
#12
S Wang, Y F Luo, J L Cao, H Zhang, X H Shi, Z Y Liang, R E Feng
Objective: To observe the histopathological changes and immunohistochemical expression of IgG4 in Riedle thyroiditis (RT) and to study the relationship between RT and IgG4-related diseases (IgG4-RD). Methods: A total of 5 RT patients were collected from the Department of Pathology, Peking Union Medical College Hospital during April 2012 to August 2014. The clinical and immunohistochemical features were analyzed in the 5 patients. Histopathologic analysis was performed on hematoxylin and eosin-stained sections...
March 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28284385/igg4-related-tubulointerstitial-nephritis
#13
REVIEW
Pingchuan Zhang, Lynn D Cornell
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fibroinflammatory disorder that can involve nearly any organ. The disorder has increasingly become known as a distinct clinical entity during the last decade. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-RD in the kidney. Many patients with IgG4-TIN are diagnosed after IgG4-RD has been recognized in other organ systems, but the kidney may also be the first or only site involved. The presenting clinical features of IgG4-TIN are most commonly kidney insufficiency, kidney mass lesion(s), or both...
March 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/28284351/is-musk-myasthenia-gravis-linked-to-igg4-related-disease
#14
Pooja Raibagkar, Judith A Ferry, John H Stone
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition that affects a wide variety of sites, including the nervous system, where it can involve the meninges or the pituitary gland, and cause perineural mass lesions. A large subset of acetylcholine receptor antibody (Ab)-negative myasthenia gravis (MG) patients has muscle-specific tyrosine kinase (MuSK) Abs, generally of the IgG4 subclass. There has not been any association found between IgG4-RD and MuSK MG yet. We report the first case of MuSK MG associated with lymphadenopathy with histopathology consistent with IgG4-RD...
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28272212/ophthalmic-manifestations-in-igg4-related-disease-clinical-presentation-and-response-to-treatment-in-a-french-case-series
#15
MULTICENTER STUDY
Mikael Ebbo, Matthieu Patient, Aurelie Grados, Matthieu Groh, Julien Desblaches, Eric Hachulla, David Saadoun, Sylvain Audia, Aude Rigolet, Benjamin Terrier, Antoinette Perlat, Constance Guillaud, Frederic Renou, Emmanuelle Bernit, Nathalie Costedoat-Chalumeau, Jean-Robert Harlé, Nicolas Schleinitz
IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series.Patients with IgG4-RD and orbital or orbital adnexa involvement included in the French multicentric IgG4-RD case-registry were identified. Only patients fulfilling "modified" comprehensive diagnostic criteria with pathological documentation were retained for the study...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28267110/cystoid-macular-edema-secondary-to-rituximab
#16
Marina Gilca, Pauline T Merrill
BACKGROUND/PURPOSE: To describe a case of bilateral macular edema appearing after treatment with rituximab. METHODS: This is a case report describing macular edema after rituximab infusions. The information was collected retrospectively through chart review. RESULTS: A 53-year-old patient known for IgG4-related disease of the face and sinuses presented with bilateral decreased visual acuity from cystoid macular edema shortly after undergoing two rituximab infusions...
March 6, 2017: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/28266140/cytokine-biomarkers-to-predict-antitumor-responses-to-nivolumab-suggested-in-a-phase-ii-study-for-advanced-melanoma
#17
Naoya Yamazaki, Yoshio Kiyohara, Hisashi Uhara, Hajime Iizuka, Jiro Uehara, Fujio Otsuka, Yasuhiro Fujisawa, Tatsuya Takenouchi, Taiki Isei, Keiji Iwatsuki, Hiroshi Uchi, Hironobu Ihn, Hironobu Minami, Hideaki Tahara
Promising anti-tumor activities of nivolumab, a fully humanized IgG4 inhibitor antibody against the programmed death-1 protein, were suggested in previous phase 1 studies. The present phase 2, single-arm study (JAPIC-CTI #111681) evaluated the anti-tumor activities of nivolumab and explored its predictive correlates in advanced melanoma patients at 11 sites in Japan. Intravenous nivolumab 2 mg/kg was given repeatedly at 3-week intervals to 35 of 37 patients enrolled from December 2011 to May 2012 until they experienced unacceptable toxicity, disease progression, or complete response...
March 7, 2017: Cancer Science
https://www.readbyqxmd.com/read/28256769/immunoglobulin-g4-related-disease-and-its-skin-manifestations
#18
REVIEW
Ryoko Takayama, Takashi Ueno, Hidehisa Saeki
Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory condition characterized by tissue infiltration with IgG4(+) plasma cells and an elevated serum IgG4 level. It usually occurs in middle-aged or older patients, with male predominance, and can involve multiple organs synchronously or metachronously. Patients with IgG4-RD generally respond to steroid therapy. IgG4-related skin lesions are typically erythematous and itchy plaques, subcutaneous nodules, or papules like prurigo nodularis located on the head and neck areas...
March 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28248797/metachronous-involvement-diagnostic-imprecision-of-serum-immunoglobulin-g4-levels-and-discordance-between-clinical-and-radiological-findings-in-immunoglobulin-g4-related-pachymeningitis-a-longitudinal-case-report
#19
Waqar Waheed, Philip Michael Skidd, Neil M Borden, Pamela C Gibson, Mohamed Ali Babi, Rup Tandan
Immunoglobulin G4-related disease is an increasingly recognized, idiopathic systemic disorder that might be associated with elevated serum IgG4 level and tissue infiltration by IgG4-positive plasma cells. We describe the clinical features and biopsy findings in a patient who presented with features suggestive of pachymeningitis and multiple cranial neuropathies. Meningeal biopsy and other laboratory studies established the diagnosis of IgG4-related hypertrophic pachymeningitis. Despite treatment with corticosteroids and mycophenolate mofetil, the patient exhibited a fluctuating progressive course, which stabilized with rituximab, although the radiological findings persisted over 2½ years of follow-up...
February 28, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28246478/eosinophilic-cholangitis-is-a-potentially-underdiagnosed-etiology-in-indeterminate-biliary-stricture
#20
Dirk Walter, Sylvia Hartmann, Eva Herrmann, Jan Peveling-Oberhag, Wolf O Bechstein, Stefan Zeuzem, Martin-Leo Hansmann, Mireen Friedrich-Rust, Jörg G Albert
AIM: To investigate presence and extent of eosinophilic cholangitis (EC) as well as IgG4-related disease in patients with indeterminate biliary stricture (IBS). METHODS: All patients with diagnosis of sclerosing cholangitis (SC) and histopathological samples such as biopsies or surgical specimens at University Hospital Frankfurt from 2005-2015 were included. Histopathological diagnoses as well as further clinical course were reviewed. Tissue samples of patients without definite diagnosis after complete diagnostic work-up were reviewed regarding presence of eosinophilic infiltration and IgG4 positive plasma cells...
February 14, 2017: World Journal of Gastroenterology: WJG
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