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IgG4 disease

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https://www.readbyqxmd.com/read/29349436/superficial-temporal-artery-aneurysm-associated-with-immunoglobulin-g4-related-disease
#1
Sosei Kuma, Tsubasa Takeshima, Takefumi Ohga, Tadahiro Nozoe, Katsuo Sueishi
A 68-year-old man was admitted because of a pulsatile mass and pain in the left temporal region, and computed tomography demonstrated the superficial temporal artery aneurysm. He underwent aneurysmectomy, and pathologic investigation revealed marked thickness of the adventitia with substantial plasmacyte infiltration. On immunoglobulin G4 (IgG4) immunohistochemistry, IgG4-positive lymphocytes were scattered in the adventitia, and biochemical tests revealed elevation of IgG4 (200 mg/dL). The case satisfied the criteria for both giant cell arteritis and IgG4-related disease (IgG4-RD)...
December 2017: Journal of Vascular Surgery Cases and Innovative Techniques
https://www.readbyqxmd.com/read/29332510/localized-autoimmune-pancreatitis-mimicking-pancreatic-cancer-case-report-and-literature-review
#2
Wen-Ling Hsu, Shu-Min Chang, Pei-Yin Wu, Chin-Chuan Chang
Autoimmune pancreatitis (AP) is a rare autoimmune pancreatic manifestation of systemic immunoglobulin G4 (IgG4)-related sclerosing disease. Distinguishing between AP and pancreatic cancer is crucial because the clinical courses, treatments, and prognoses of these two disease entities are quite different. We herein report a case involving a 52-year-old man with subacute epigastralgia who visited our hospital for evaluation of a suspicious pancreatic mass found during esophagogastroduodenoscopy. Enhanced computed tomography (CT) revealed an enlarged lesion in the pancreatic head with encasement of hepatic vessels...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29329570/exophthalmos-in-a-young-woman-with-no-graves-disease-a-case-report-of-igg4-related-orbitopathy
#3
Annamaria Erdei, Zita Steiber, Csaba Molnar, Ervin Berenyi, Endre V Nagy
BACKGROUND: Immunoglobulin G4-related disease (IgG4-rd) is characterized by lymphoplasmacytic infiltration and tissue fibrosis. Orbital manifestations of IgG4-rd may include unilateral or bilateral proptosis, cicatricial extraocular muscle myopathy, orbital inflammation and pain which may mimic ophthalmic Graves' disease. CASE PRESENTATION: A 25-year-old woman has been referred to the endocrinology clinic, 4 months after delivery, with suspected Graves' orbitopathy...
January 12, 2018: BMC Ophthalmology
https://www.readbyqxmd.com/read/29324858/mapping-the-geographical-distribution-of-podoconiosis-in-cameroon-using-parasitological-serological-and-clinical-evidence-to-exclude-other-causes-of-lymphedema
#4
Kebede Deribe, Amuam Andrew Beng, Jorge Cano, Abdel Jelil Njouendo, Jerome Fru-Cho, Abong Raphael Awah, Mathias Esum Eyong, Patrick W Chounna Ndongmo, Emanuele Giorgi, David M Pigott, Nick Golding, Rachel L Pullan, Abdisalan M Noor, Fikre Enquselassie, Christopher J L Murray, Simon J Brooker, Simon I Hay, Peter Enyong, Melanie J Newport, Samuel Wanji, Gail Davey
BACKGROUND: Podoconiosis is a non-filarial elephantiasis, which causes massive swelling of the lower legs. It was identified as a neglected tropical disease by WHO in 2011. Understanding of the geographical distribution of the disease is incomplete. As part of a global mapping of podoconiosis, this study was conducted in Cameroon to map the distribution of the disease. This mapping work will help to generate data on the geographical distribution of podoconiosis in Cameroon and contribute to the global atlas of podoconiosis...
January 2018: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/29324739/mansonella-perstans-microfilaremic-individuals-are-characterized-by-enhanced-type-2-helper-t-and-regulatory-t-and-b-cell-subsets-and-dampened-systemic-innate-and-adaptive-immune-responses
#5
Manuel Ritter, Winston Patrick Chounna Ndongmo, Abdel Jelil Njouendou, Nora Nganyewo Nghochuzie, Lucy Cho Nchang, Dizzle Bita Tayong, Kathrin Arndts, Norman Nausch, Marc Jacobsen, Samuel Wanji, Laura E Layland, Achim Hoerauf
The filarial nematode Mansonella perstans is endemic throughout Africa, northern South America and the Caribbean. Interestingly, M. perstans-infected individuals present no distinct clinical picture associated with certain pathology. Due to its relatively silent nature, research on this tropical disease has been neglected, especially M. perstans-driven immune responses. A hindrance in obtaining data on M. perstans-specific responses has been the inability to obtain adult worms since their habitats in serous cavities are difficult to access...
January 11, 2018: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/29323079/immunoglobulin-g4-related-tubulointerstitial-nephritis-a-not-to-be-missed-diagnosis
#6
Smita Mary Matthai, Anjali Mohapatra, Raiyani Palak, Gopal Basu
Immunoglobulin G4-related tubulointerstitial nephritis (IgG4-TIN) is a newly recognized clinicopathological entity characterized by a dense interstitial infiltrate of IgG4-positive plasma cells accompanied by fibrosis and obliterative phlebitis causing acute or chronic renal dysfunction amenable to corticosteroid therapy. IgG4-TIN is the dominant manifestation of renal involvement in IgG4-related disease (IgG4-RD) which is a novel, immune-mediated, fibroinflammatory and multiorgan disorder. We describe a case of IgG4-TIN with isolated renal involvement in an elderly male patient with poor response to corticosteroid therapy...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29313399/localized-intrahepatic-igg4-related-sclerosing-cholangitis-igg4-sc-as-an-additional-type-of-igg4-sc-a-systematic-analysis-of-12-cases
#7
Zhichao Mo, Kuvaneshan Ramen, Yunfeng Shan, Mengtao Zhou, Xiangwu Zheng, Xiuling Wu, Zhengping Yu, Xiaoke Ji, Qiyu Zhang, Qiqiang Zeng
OBJECTIVES: IgG4-related sclerosing cholangitis (IgG4-SC), a recently defined disease entity, has been classified into four types based on the stricture regions revealed by cholangiography. However, localized intrahepatic IgG4-SC is not included into the classification. This study aimed to analyze and characterize localized intrahepatic IgG4-SC and justify the inclusion of this type into the classification. METHODS: PubMed and Embase were searched for studies published from March 2001 to June 2017 reporting localized intrahepatic IgG4-SC...
January 9, 2018: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/29313220/pemphigus-a-comprehensive-review-on-pathogenesis-clinical-presentation-and-novel-therapeutic-approaches
#8
REVIEW
Robert Pollmann, Thomas Schmidt, Rüdiger Eming, Michael Hertl
Pemphigus is a group of rare, potentially devastating autoimmune diseases of the skin and mucous membranes with high morbidity and potentially lethal outcome. The major clinical variant, pemphigus vulgaris (PV) is caused by a loss of intercellular adhesion of epidermal keratinocytes which is induced by IgG autoantibodies against components of desmosomes. Specifically, IgG against the desmosomal adhesion proteins, desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1), preferentially target their ectodomains which are presumably critical for the transinteraction and signalling function of these adhesion molecules...
January 8, 2018: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29311390/hepatitis-b-virus-reactivation-during-steroid-treatment-of-igg4-related-disease
#9
Alexandra Shingina, Robert Puckrin, Aliya Gulamhusein
No abstract text is available yet for this article.
December 27, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/29310344/polymyositis-with-elevated-serum-igg4-levels-and-abundant-igg4-plasma-cell-infiltration-a-case-report-and-literature-review
#10
REVIEW
Ryusuke Anan, Mitsuhiro Akiyama, Yuko Kaneko, Jun Kikuchi, Kazuko Suzuki, Shiro Matsubara, Tsutomu Takeuchi
INTRODUCTION: Polymyositis (PM) is a type of autoimmune, inflammatory myopathy. IgG4-related disease (IgG4-RD) is a recently recognized disease entity characterized by elevated serum IgG4 levels and IgG4 plasma-cell infiltration of various organs. However, several reports have described cases of other diseases that present with those features, suggesting the importance of careful differential diagnosis. Herein, we report the first case of PM with elevated serum IgG4 levels and IgG4 plasma cells in the muscles, mimicking IgG4-RD...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29298790/a-case-of-igg4oligoarthritis-mimicking-psoriatic-arthritis
#11
Ziad Farah, Neil Mo
IgG4-related disease (IgG4-RD) is a rare but rapidly emerging immune-mediated fibroinflammatory condition that can affect almost any organ. It is typically associated with involvement of organs such as lymph nodes, submandibular glands, orbits, periaortic region and pancreas. However, IgG4-RD presenting primarily as an inflammatory arthritis is much less recognised. We present a rare case of IgG4-RD mimicking psoriatic arthritis. In spite of normal circulating IgG4 plasma levels, a clinical index of suspicion was required to obtain a histopathological diagnosis...
January 3, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29297990/video-assisted-thoracoscopic-surgery-can-help-enable-the-complete-resection-of-a-mediastinal-tumor-caused-by-immunoglobulin-g4-related-disease-and-avoid-the-need-for-postoperative-medication-a-case-report
#12
Tsuyoshi Uchida, Hirochika Matsubara, Satoshi Nagasaka, Satsuki Kina, Tomofumi Ichihara, Hiroyasu Matsuoka, Hiroyuki Nakajima
Immunoglobulin (Ig) G4-related disease has various clinical signs and symptoms, and steroidal therapy with corticosteroids has been found to be effective for treatment. Few cases of IgG4-related disease associated with paravertebral tumor have been reported, and there have been no reports on complete resection of such a tumor. Here, we report a case of IgG4-related disease associated with a paravertebral tumor that was successfully resected without the need for postoperative medication. An 84-year-old woman was admitted to our hospital with a paravertebral tumor...
January 3, 2018: Asian Journal of Endoscopic Surgery
https://www.readbyqxmd.com/read/29296751/a-novel-cxcr4-antagonist-igg1-antibody-pf-06747143-for-the-treatment-of-hematologic-malignancies
#13
Shu-Hui Liu, Yin Gu, Bernadette Pascual, Zhengming Yan, Max Hallin, Cathy Zhang, Conglin Fan, Wenlian Wang, Justine Lam, Mary E Spilker, Rolla Yafawi, Eileen Blasi, Brett Simmons, Nanni Huser, Wei-Hsien Ho, Kevin Lindquist, Thomas-Toan Tran, Jyothirmayee Kudaravalli, Jing-Tyan Ma, Gretchen Jimenez, Ishita Barman, Colleen Brown, Sherman Michael Chin, Maria J Costa, David Shelton, Tod Smeal, Valeria R Fantin, Flavia Pernasetti
The chemokine receptor CXCR4 is highly expressed and associated with poor prognosis in multiple malignancies. Upon engagement by its ligand, CXCL12, CXCR4 triggers intracellular signaling pathways that control trafficking of cells to tissues where the ligand is expressed, such as the bone marrow (BM). In hematologic cancers, CXCR4-driven homing of malignant cells to the BM protective niche is a key mechanism driving disease and therapy resistance. We developed a humanized CXCR4 immunoglobulin G1 (IgG1) antibody (Ab), PF-06747143, which binds to CXCR4 and inhibits CXCL12-mediated signaling pathways, as well as cell migration...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29289265/utility-of-serum-igg4-levels-in-a-multiethnic-population
#14
Ruyu Qi, Luke Y C Chen, Sujin Park, Robert Irvine, Michael A Seidman, John T Kelsall, David Collins, Vivian Yin, Graham W Slack, Andre Mattman, Eric Lam, Mollie N Carruthers
BACKGROUND: IgG4-related disease (IgG4-RD) is a recently recognized condition defined by characteristic histopathologic findings in affected organs. Serum IgG4 concentration is often but not always elevated. The sensitivity and specificity of serum IgG4 vary greatly across studies and has been anecdotally associated to ethnicity. Our study was conducted to investigate the difference in serum IgG4 levels between Asian and non-Asian patients with IgG4-RD. METHODS: This is a single-center retrospective study of 26 Asian and 10 non-Asian patients with histologically confirmed IgG4-RD...
January 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29288833/lacrimal-gland-abscess-in-a-child-as-a-rare-manifestation-of-igg4-related-disease
#15
Edward L Raab, Hamideh S Moayedpardazi, Steven M Naids, Alan H Friedman, Murray A Meltzer
Dacryoadenitis is unusual in children, and noninfectious causes can be manifestations of serious undiagnosed systemic conditions. This report brings further attention to this rarely-encountered condition and emphasizes its association with IgG4-related disease, a group of inflammatory disorders of high current interest whose spectrum of manifestations continues to evolve. The pediatric ophthalmologist could be in the position of identifying a patient's serious and previously unappreciated illness.
December 27, 2017: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/29288039/immunoglobulin-g4-related-chronic-rhinosinusitis-a-pitfall-in-the-differential-diagnosis-of-granulomatosis-with-polyangiitis-rosai-dorfman-disease-and-fungal-rhinosinusitis
#16
Yingshi Piao, Yuan Zhang, Changli Yue, Chengshuo Wang, Luo Zhang
Immunoglobulin G4 (IgG4)-related chronic rhinosinusitis (CRS) has recently been proposed to be a new clinical entity of nasal disease, with no consensually agreed criteria for diagnosis. Moreover, the pathological features if IgG4-related CRS often overlap with other sinonasal inflammatory and autoimmune diseases such as Granulomatosis with Polyangiitis (GPA), Rosai-Dorfman disease (RDD) and Fungal Rhinosinusitis (FRS). We aimed to explore the specific similarities and differences in clinicopathologic features between IgG4-related CRS, and GPA, RDD and FRS, in order that these conditions can be diagnosed more accurately...
December 26, 2017: Human Pathology
https://www.readbyqxmd.com/read/29285637/lymphomas-in-igg4-related-disease-clinicopathologic-features-in-a-western-population
#17
Jacob R Bledsoe, Zachary S Wallace, John H Stone, Vikram Deshpande, Judith A Ferry
Lymphomas that occur in the setting of IgG4-related disease (IgG4-RD) are uncommon. Most reported cases derive from Asia and are MALT lymphomas occurring in orbital IgG4-RD. The spectrum of lymphomas among IgG4-RD patients in the Western world remains poorly defined. The aim of this study was to report our experience with lymphomas occurring in IgG4-RD. Eight cases were identified from the pathology and consultation files. The median age was 61 years (range 22-68) at IgG4-RD diagnosis and 63.5 years (range 33-79) at lymphoma diagnosis, with a M:F ratio of 4:4...
December 28, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29285599/computed-tomography-and-magnetic-resonance-imaging-of-peripelvic-and-periureteric-pathologies
#18
REVIEW
Ekim Gumeler, Mehmet Ruhi Onur, Ali Devrim Karaosmanoglu, Mustafa Ozmen, Deniz Akata, Musturay Karcaaltincaba
Peripelvic and periureteric areas are frequently overlooked in the imaging evaluations of the urinary system on computed tomography and magnetic resonance imaging. Several neoplastic and non-neoplastic disorders and diseases including lipomatosis, angiomyolipoma and angiolipomatous proliferation, vascular lesions, lymphangiomatosis, Rosai-Dorfman disease, Erdheim-Chester disease, extramedullary hematopoiesis, IgG4-related disease, lymphoma, mesenchymal tumors, trauma, and Antopol-Goldman lesion may involve these areas...
December 28, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/29279552/nephrotic-syndrome-during-the-tapering-of-oral-steroids-after-pathological-diagnosis-of-kimura-disease-from-a-lacrimal-gland-mass-case-report-and-review-of-10-japanese-patients
#19
Toshihiko Matsuo, Takehiro Tanaka, Masaru Kinomura
A 42-year-old man with eosinophilia and high serum immunoglobulin E (IgE) developed a lacrimal gland mass on the left side. Excisional biopsy revealed hyperplasia of lymphoid follicles, and infiltration with lymphocytes and eosinophils around lacrimal gland acini, leading to the pathological diagnosis of Kimura disease. IgE-positive cells were mainly found along follicular dendritic cells, and a small number of IgG4-positive cells was present. One month after oral prednisolone was started at 40 mg daily and tapered to 10 mg daily, he developed lower leg edema on both sides and marked proteinuria (10...
2017: Journal of Clinical and Experimental Hematopathology: JCEH
https://www.readbyqxmd.com/read/29279491/igg4-related-disease-a-systemic-disease-that-deserves-attention-regardless-of-one-s-subspecialty
#20
Hideaki Hamano, Eiji Tanaka, Nobukazu Ishizaka, Shigeyuki Kawa
IgG4-related disease (IgG4-RD) is an inflammatory condition characterized by a high serum IgG4 concentration and the abundant infiltration of lymphocytes and IgG4-positive plasma cells in the tissue, as well as spatial (diverse clinical manifestations) and temporal (the possibility of recurrence) multiplicities. Since the initial documentation of IgG4-related disease in patients with autoimmune pancreatitis in 2001, a growing body of evidence has been accumulating to suggest that various-virtually all-organs can be affected by IgG4-RD...
December 27, 2017: Internal Medicine
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