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IgG4 disease

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https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#1
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29133623/immu-140-a-novel-sn-38-antibody-drug-conjugate-targeting-hla-dr-mediates-dual-cytotoxic-effects-in-hematological-cancers-and-malignant-melanoma
#2
Thomas M Cardillo, Serengulam V Govindan, Maria B Zalath, Diane L Rossi, Yang Wang, Chien-Hsing Chang, David M Goldenberg
HLA-DR is a member of the MHC class II antigen family expressed on hematological and solid tumors.  Antibodies directed against HLA-DR have demonstrated some clinical success, but toxicities limited development.  IMMU-140 is an anti-HLA-DR antibody-drug conjugate comprised of the active metabolite of irinotecan, SN-38, conjugated to a humanized anti-HLA-DR IgG4 antibody (IMMU-114); the IgG4 naked antibody is devoid of immune functions. Our aim was to determine if SN-38, the metabolite of a drug not commonly used in hematopoietic cancers, would be effective and safe when targeted to HLA-DR-expressing tumors...
November 13, 2017: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/29130507/neurofascin-155-igg4-neuropathy-pathophysiological-insights-spectrum-of-clinical-severity-and-response-to-treatment
#3
Nidhi Garg, Susanna B Park, Con Yiannikas, Steve Vucic, James Howells, Yu-Ichi Noto, Emily K Mathey, John D Pollard, Matthew C Kiernan
INTRODUCTION: Sensorimotor neuropathy associated with IgG4 antibodies to neurofascin-155 (NF155) was recently described. The clinical phenotype is typically associated with young onset, distal weakness and in some cases, tremor. METHODS: From a consecutive cohort of 55 patients diagnosed with chronic inflammatory demyelinating polyneuropathy, screening for anti-NF155 antibodies was undertaken. Patients underwent clinical assessment, diagnostic neurophysiology, including peripheral axonal excitability studies and nerve ultrasound...
November 11, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/29125188/the-mouse-passive-transfer-model-of-musk-myasthenia-gravis-disrupted-musk-signaling-causes-synapse-failure
#4
REVIEW
Nazanin Ghazanfari, Sofie Trajanovska, Marco Morsch, Simon X Liang, Stephen W Reddel, William D Phillips
While the majority of myasthenia gravis patients express antibodies targeting the acetylcholine receptor, the second most common cohort instead displays autoantibodies against muscle-specific kinase (MuSK). MuSK is a transmembrane tyrosine kinase found in the postsynaptic membrane of the neuromuscular junction. During development, MuSK serves as a signaling hub, coordinating the alignment of the pre- and postsynaptic components of the synapse. Adult mice that received repeated daily injections of IgG from anti-MuSK(+) myasthenia gravis patients developed muscle weakness, associated with neuromuscular transmission failure...
November 10, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29125145/the-2017-doyne-lecture-the-orbit-as-a-window-to-systemic-disease
#5
REVIEW
A A McNab
A very large number of disorders affect the orbit, and many of these occur in the setting of systemic disease. This lecture covers selected aspects of orbital diseases with systemic associations in which the author has a particular clinical or research interest. Spontaneous orbital haemorrhage often occurs in the presence of bleeding diatheses. Thrombosis of orbital veins and ischaemic necrosis of orbital and ocular adnexal tissues occur with thrombophilic disorders, vasculitis, and certain bacterial and fungal infections...
November 10, 2017: Eye
https://www.readbyqxmd.com/read/29117468/zoonotic-helminth-exposure-and-risk-of-allergic-diseases-a-study-of-two-generations-in-norway
#6
Nils Oskar Jõgi, Cecilie Svanes, Silver Peeter Siiak, Erin Logan, John W Holloway, Jannicke Igland, Ane Johannessen, Michael Levin, Francisco Gomez Real, Vivi Schlunssen, William G C Horsnell, Randi J Bertelsen
BACKGROUND: Animal and human studies indicate that definitive host helminth infections may confer protection from allergies. However, zoonotic helminths, such as Toxocara species (spp.), have been associated with increased allergies. OBJECTIVE: We describe the prevalence of Toxocara spp. and Ascaris spp. seropositivity, and associations with allergic diseases and sensitisation, in two generations in Bergen, Norway. METHODS: Serum levels of total IgG4, anti-Toxocara spp...
November 8, 2017: Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29110571/igg4-related-disease
#7
Daniel Blockmans
No abstract text is available yet for this article.
November 7, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29105322/clinical-characteristics-and-outcomes-of-61-patients-with-chronic-periaortitis-including-igg4-related-and-non-igg4-related-cases
#8
In Young Kim, Yeong Hee Eun, Hyemin Jeong, Taek Kyu Park, Hyungjin Kim, Jaejoon Lee, Shin Yi Jang, Jung-Sun Kim, Eun-Mi Koh, Duk-Kyung Kim, Hoon-Suk Cha
AIM: Chronic periaortitis (CP) is a disease characterized by a fibro-inflammatory periaortic cuff and adventitia-predominant fibrosis. CP encompasses idiopathic retroperitoneal fibrosis and inflammatory abdominal aortic aneurysm (AAA), and recent studies have documented overlap between CP and immunoglobulin G4-related disease (IgG4-RD). This study aimed to investigate clinical characteristics and treatment outcomes of patients with CP. METHOD: CP patients were identified by retrospective review of 1245 patients with International Classification of Diseases 10th edition code of aortitis or aortic disease...
November 3, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29101259/deciphering-the-biology-of-igg4-related-disease-specific-antigens-and-disease
#9
EDITORIAL
Debashis Haldar, Gideon M Hirschfield
No abstract text is available yet for this article.
November 3, 2017: Gut
https://www.readbyqxmd.com/read/29099539/igg4-related-disease-in-thymus-a-very-rare-case-of-chronic-fibrosis-mimicking-sarcoidosis
#10
Sara Simonetti, Noelia Pérez Muñoz, Josefa López Vivancos, Lluís Sanchez Sitjes, Javier Cesar Herranz Pérez, Nelson Leal Bohorquez, José Antonio Maestre Alcacer, Inessa Koptseva de García, Miguel Ángel Carrasco García
IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated chronic fibroinflammatory condition, with unclear certain etiology. It is morphologically characterized by storiform fibrosis, dense IgG4-positive lymphoplasmacytic infiltrate, and obliterative phlebitis. It was recognized as a systemic condition as recently as 2003. IgG4-RD has been described in virtually every organ, forming sclerosing masses, and often mimicking tumors. Clinically, patients present unspecific symptoms and this condition is often recognized incidentally...
October 28, 2017: Tumori
https://www.readbyqxmd.com/read/29093590/-autoimmune-pancreatitis-type-1-a-case-report
#11
Álvaro Bellido-Caparó, Jorge Espinoza-Ríos, Víctor Aguilar, Carlos García, José Luis Pinto, Martin Tagle Arróspide, Alejandro Bussalleu Rivera
Autoimmune pancreatitis type 1 (AIP) is the pancreatic manifestation of IgG4-related disease. The most frequent presentation of AIP is with obstructive jaundice. For definite diagnosis of type 1 Autoimmune pancreatitis international consensus diagnosis criteria (ICDC) for AIP are used. ICDC criteria include pancreatic parenchymal imaging, ductal imaging, serology, other organ involvement, histology, and response to steroid. We report a 52-years-old woman with rheumatoid arthritis without treatment presented with two months of abdominal pain in up-right quadrant with moderate intensity...
July 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/29093426/potential-importance-of-a-histopathological-analysis-in-thyroidal-diseases-with-high-serum-igg4-levels
#12
Hiroshi Nomoto, Hideaki Miyoshi, Akinobu Nakamura, So Nagai, Chikara Shimizu, Tatsuya Atsumi
No abstract text is available yet for this article.
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29093307/warthin-like-papillary-thyroid-carcinoma-with-immunoglobulin-g4-positive-plasma-cells-possibly-related-to-hashimoto-s-thyroiditis
#13
Mitsuyoshi Hirokawa, Eijun Nishihara, Nami Takada, Miyoko Higuchi, Masumi Kotakemori, Toshitetsu Hayashi, Akira Miyauchi
Hashimoto's thyroiditis with heavy lymphoplasmacytic infiltration is a common comorbidity of immunoglobulin G4 (IgG4)-related thyroiditis and Warthin-like papillary thyroid carcinoma (WL-PTC). We hypothesized that WL-PTC may have a strong association with IgG4-related thyroiditis. To validate this hypothesis, we clinically and immunohistochemically studied 17 WL-PTC cases. Fourteen patients (82.4%) had anti-thyroglobulin antibody and were confirmed to have Hashimoto's thyroiditis through microscopic analysis...
November 2, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/29072949/igg4-related-disease-a-reminder-for-practicing-pathologists
#14
REVIEW
Steven C Weindorf, John Karl Frederiksen
IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic infiltrates rich in immunoglobulin (Ig) G4(+) plasma cells. Initially characterized as a form of autoimmune pancreatitis, the distinctive histopathology of IgG4-RD has now been described in almost every organ system. However, because the clinical manifestations of IgG4-RD are diverse and nonspecific, the disease may go unsuspected until a biopsy or resection specimen is obtained to diagnose a presumed malignancy...
November 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/29067928/multi-organ-igg4-related-disease-demystifying-the-diagnostic-enigma
#15
S Bhardwaj, S Goyal, A K Yadav, A Goyal
IgG4-related disease (IgG4-RD) is a multisystemic mass forming immune-mediated disease entity, commonly creating confusion and diagnostic challenges. We present a case of a 25-year-old female who presented with bilateral orbital masses, lymphadenopathy, paraspinal and renal masses, which clinicoradiologically simulated lymphoma. The lymph node biopsy revealed interfollicular sheets of plasma cells creating confusion with Castleman's disease and marginal zone lymphoma. The orbital biopsy revealed ductular destruction, periductular plasma cells, and fibrosis, mimicking Sjogren's syndrome and Castleman's disease...
October 23, 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/29067257/a-study-of-serum-igg4-levels-in-the-clinical-metamorphosis-of-autoimmune-thyroid-disease
#16
Yotsapon Thewjitcharoen, Sirinate Krittiyawong, Sriurai Porramatikul, Ekgaluck Wanothayaroj, Nampetch Lekpittaya, Jirapat Jeamjiraprasert, Soontaree Nakasatien, Thep Himathongkam
BACKGROUND: Measurement of serum IgG4 had been suggested to distinguish the unique subtypes of autoimmune thyroid disease (AITD) which demonstrated patterns of fluctuating between hyperthyroidism and hypothyroidism. However, the clinical utility of serum IgG4 measurement is inconclusive due to few studies having addressed these unusual patients compared with the specificity of serum IgG4 in healthy patients. AIM: To investigate whether elevated serum IgG4 levels could be used as a marker to identify fluctuating AITD patients...
June 2017: Journal of Clinical & Translational Endocrinology
https://www.readbyqxmd.com/read/29063242/clinical-spectrum-and-igg-subclass-analysis-of-anti-myelin-oligodendrocyte-glycoprotein-antibody-associated-syndromes-a-multicenter-study
#17
Sara Mariotto, Sergio Ferrari, Salvatore Monaco, Maria Donata Benedetti, Kathrin Schanda, Daniela Alberti, Alessia Farinazzo, Ruggero Capra, Chiara Mancinelli, Nicola De Rossi, Roberto Bombardi, Luigi Zuliani, Marco Zoccarato, Raffaella Tanel, Adriana Bonora, Marco Turatti, Massimiliano Calabrese, Alberto Polo, Antonino Pavone, Luisa Grazian, GianPietro Sechi, Elia Sechi, Daniele Urso, Rachele Delogu, Francesco Janes, Luciano Deotto, Morena Cadaldini, Maria Rachele Bianchi, Gaetano Cantalupo, Markus Reindl, Alberto Gajofatto
Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) recently emerged as a potential biomarker in patients with inflammatory demyelinating diseases of the central nervous system. We here compare the clinical and laboratory findings observed in a cohort of MOG-Ab seropositive and seronegative cases and describe IgG subclass analysis results. Consecutive serum samples referred to Verona University Neuropathology Laboratory for aquaporin-4 (AQP4)-Ab and/or MOG-Ab testing were analysed between March 2014 and May 2017...
October 23, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29063068/immunoglobulin-g4-related-kidney-disease-pathogenesis-diagnosis-and-treatment
#18
REVIEW
Ke Zheng, Fei Teng, Xue-Mei Li
Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized clinical entity that often involves multiple organs; it is characterized by high levels of serum immunoglobulin G4 (IgG4), dense infiltration of IgG4+ cells, and storiform fibrosis. Cellular immunity, particularly T cell-mediated immunity, has been implicated in the pathogenesis of IgG4-RD. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulonephropathy (MGN), and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis, prostatitis, or ureter inflammation...
September 2017: Chronic Dis Transl Med
https://www.readbyqxmd.com/read/29061247/neurologic-features-of-immunoglobulin-g4-related-disease
#19
REVIEW
Mahmoud AbdelRazek, John H Stone
Immunoglobulin G4-related disease (IgG4-RD) can involve nearly any organ system, including the central and peripheral nervous systems. IgG4 antibodies are not known to play a primary etiologic role in disease. IgG4-RD must be distinguished from a growing number of immune-mediated conditions in which IgG4 autoantibodies contribute directly to pathophysiology. The most common neurologic features of IgG4-RD result from disease in the orbits, pachymeninges, and substance of the pituitary gland and stalk, as well as a perineuropathy that can involve peripheral or cranial nerves...
November 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29053038/igg4-related-orbital-disease-masquerading-as-thyroid-eye-disease-vice-versa-or-both
#20
Joyce Khandji, Ashley A Campbell, Alison B Callahan, Portia Sirinek, Michael Kazim
A 40 year-old male presented after one year of unilateral, progressive, steroid-responsive, orbital inflammatory disease causing proptosis, extraocular muscle (EOM) restriction, and compressive optic neuropathy. The development of anti-thyroidal antibodies prompted the diagnosis of thyroid eye disease (TED); however, the prolonged active phase, remarkable reversibility of ophthalmic features with high-dose corticosteroids, unilaterally of disease, uncharacteristic EOM involvement (including both obliques), and the absence of autoimmune thyroid disease provoked consideration of alternative diagnoses...
October 20, 2017: Orbit
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