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IgG4 disease

Shinya Fujie, Hiroyuki Matsubayashi, Hirotoshi Ishiwatari, Hiromasa Hazama, Takaaki Ito, Keiko Sasaki, Hiroyuki Ono
A 70-year-old man was referred to our hospital with exacerbation of diabetes. His blood tests showed elevated levels of serum IgG4 and HbA1c. Computed tomography of the pancreatic body demonstrated a weakly enhanced mass, 2 cm in size, with indistinct borders. Magnetic resonance cholangiopancreatography revealed a narrowing of the main pancreatic duct (MPD) at the pancreatic body, a markedly dilated upstream duct, and a slightly dilated downstream duct. Endoscopic ultrasonography demonstrated an iso-hypoechoic heterogeneous mass, protruding and spreading in the pancreatic duct...
March 2018: Journal of Gastrointestinal and Liver Diseases: JGLD
Gonçalo Pestana, Teresa Pinho, Catarina Costa, Pedro Bernardo Almeida, Paulo Pinho, M Júlia Maciel
No abstract text is available yet for this article.
March 15, 2018: Revista Española de Cardiología
Jae-Il Lee, Yun-Jung Choi, Hi-Jung Park, Kyeong Cheon Jung, Seong Hoe Park
Antibody formation against therapeutic agents, such as tumor necrosis factor inhibitors and Factor VIII, that leads to treatment failure has become a major challenge in the treatment of rheumatoid arthritis and hemophilia. It is well known that anti-CD154 antibodies have the highest potential to inhibit these types of adverse immune responses. Nevertheless, the formation of thromboemboli is the major hurdle in the clinical application of these anti-CD154 blocking antibodies. For this, we attempted to derive an idea as to how this major complication can be eliminated...
March 16, 2018: Biochemical and Biophysical Research Communications
Eva Roos, Lowiek M Hubers, Robert J S Coelen, Marieke E Doorenspleet, Niek de Vries, Joanne Verheij, Ulrich Beuers, Thomas M van Gulik
BACKGROUND: Distinguishing perihilar cholangiocarcinoma (PHC) from benign forms of sclerosing cholangitis affecting the hilar bile ducts is challenging, since histological confirmation of PHC is difficult to obtain and accurate non-invasive diagnostic tests are not available. IgG4-associated cholangitis (IAC), an imitator of PHC, may present with clinical and radiographical signs of PHC. IAC can be accurately diagnosed with a novel qPCR test. The aim of this study was to investigate the incidence and long-term activity of IAC in patients resected for PHC in a single tertiary center over a period of 30 years...
March 16, 2018: American Journal of Gastroenterology
Mitsuhiro Akiyama, Tsutomu Takeuchi
IgG4-related disease is a heterogeneous immune-mediated fibroinflammatory condition that can affect every single organ. This disease is more prevalent in the elderly (the mean age of patients is above 60 years) and the prevalence rate is estimated to be over 4.6 per 100,000 population. Before making a diagnosis, the exclusion of malignancies, lymphoma, anti-neutrophil cytoplasmic antibody-associated vasculitis, multicentric Castleman disease, and other mimickers is crucial for appropriate treatment. Broad management guidelines have been published emphasizing the need for prompt treatment and the use of glucocorticoids as first-line drug therapy for induction of remission...
March 15, 2018: Drugs & Aging
Kenji Nishida, Yuka Gion, Mai Takeuchi, Takehiro Tanaka, Tatsuki R Kataoka, Tadashi Yoshino, Yasuharu Sato
Immunoglobulin G4 (IgG4)-related disease is characterized by elevated serum IgG4 levels and increased numbers of IgG4-positive cells. However, its pathogenesis is not fully understood. We previously suggested that mast cells may play an important role in IgG4-related disease. In this study, we confirmed the characteristics of mast cells in IgG4-related lymphadenopathy by using immunohistochemistry and dual immunofluorescence. We analyzed 23 cases of IgG4-related lymphadenopathy and compared them with 23 cases of non-specific lymphoid hyperplasia...
March 15, 2018: Scientific Reports
Yasuhiro Kinugawa, Takeshi Uehara, Kazuyuki Matsuda, Yukihiro Kobayashi, Tomoyuki Nakajima, Hideaki Hamano, Shigeyuki Kawa, Kayoko Higuchi, Noriko Hosaka, Satoshi Shiozawa, Hiroki Ishigame, Toshitsugu Nakamura, Yasuhiro Maruyama, Koh Nakazawa, Masato Nakaguro, Kenji Sano, Hiroyoshi Ota
The relationship between methylation abnormality and autoimmune pancreatitis (AIP)-a representative IgG4-related disease-has not yet been elucidated. We identified SKI might have a significant methylation abnormality in AIP through methylation array analysis using the Illumina Infinium Human Methylation 450K BeadChip array, and investigated the relationship of SKI with AIP clinicopathological features. The methylation rate of SKI was assessed by quantitative SYBR green methylation-specific PCR, and the degree of SKI expression in tissue specimens was assessed by immunohistochemistry in 10 AIP cases, 14 cases of obstructive pancreatitis area in pancreatic ductal adenocarcinoma (PDA) without a history of AIP, and 9 normal pancreas (NP) cases...
March 6, 2018: Pathology, Research and Practice
Atsushi Nagayasu, Satoshi Kubo, Kazuhisa Nakano, Shingo Nakayamada, Shigeru Iwata, Ippei Miyagawa, Shunsuke Fukuyo, Kazuyoshi Saito, Yoshiya Tanaka
An 81-year-old man was admitted with bilateral pleural effusion. A clinical examination showed lymphocytic pleura effusion and elevated serum IgG4 levels, so that IgG4-related disease was suggested, whereas tuberculous pleurisy was suspected because of high adenosine deaminase (ADA) levels in the pleural effusion. A surgical pleural biopsy revealed that there were large numbers of IgG4-positive cells and IgG4/IgG positive cell ratio exceeded 40% in several sites. Accordingly, we diagnosed IgG4-related pleuritis and treated with the patient with glucocorticoid therapy...
March 9, 2018: Internal Medicine
Shounak Majumder, Sonmoon Mohapatra, Ryan J Lennon, Guilherme Piovezani Ramos, Neil Postier, Ferga Gleeson, Michael J Levy, Randall K Pearson, Bret T Petersen, Santhi S Vege, Suresh T Chari, Mark Topazian, Thomas E Witzig
BACKGROUND & AIMS: Immunoglobulin G4-related disease (IgG4-RD), a multi-organ fibro-inflammatory syndrome, typically responds to steroids. However, some cases are steroid resistant, and pancreaticobiliary IgG4-RD commonly relapses after steroid withdrawal. Rituximab induces remission of IgG4-RD, but the need for and safety of maintenance rituximab treatment are unknown. We compared outcomes of patients with pancreaticobiliary IgG4-RD treated with or without maintenance rituximab therapy...
March 8, 2018: Clinical Gastroenterology and Hepatology
Kyoko Otani, Dai Inoue, Tomoo Itoh, Yoh Zen
AIMS: The diagnosis of IgG4-related disease (IgG4-RD) requires a multidisciplinary approach, in which histology plays an important role. Although a diagnosis was previously established using surgically resected specimens, there is increasing clinical demand to diagnose this systemic condition by biopsies. The present study aimed to elucidate how useful transbronchial lung biopsies (TBLBs) are for this diagnostic purpose. METHODS AND RESULTS: The study cohort consisted of 20 consecutive patients diagnosed with IgG4-RD in other organs who underwent TBLBs for potential pulmonary involvement...
March 11, 2018: Histopathology
John M Kelso
Patients often seek opinions from allergists regarding unconventional testing for adverse reactions to foods. These tests include flow cytometry to measure the change in white blood cell volumes after incubation with foods, measurement of serum IgG or IgG4 antibodies directed against foods, intradermal provocation-neutralization with food allergens, hair analysis, electrodermal testing, and applied kinesiology. In some cases, although the laboratory methods may be valid, there are no studies showing correlation with disease...
March 2018: Journal of Allergy and Clinical Immunology in Practice
M Cecilia Berin, Alexander Grishin, Madhan Masilamani, Donald Y Leung, Scott H Sicherer, Stacie M Jones, A Wesley Burks, Alice K Henning, Peter Dawson, Joanna Grabowska, Charuta Agashe, Wendy F Davidson, Robert A Wood, Hugh A Sampson
BACKGROUND: Egg allergy is phenotypically heterogeneous. A subset of egg allergic individuals can tolerate egg in an extensively heated form. Inclusion of baked-egg (BE) into their diet accelerates resolution of egg allergy. Conversely, BE reactivity is associated with persistent disease. The immune basis of this clinical heterogeneity is unknown. OBJECTIVES: To study egg-specific antibody, basophil, and T cell responses in children with reactivity or tolerance to BE...
March 5, 2018: Journal of Allergy and Clinical Immunology
A Chawla, S Shikhare, D Singh
No abstract text is available yet for this article.
January 2018: Indian Journal of Nephrology
N Yoshimoto, H Ujiie, M Zheng, H Iwata, H Kosumi, H Hata, H Shimizu
Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering skin disease, characterized by tense bullae and urticarial erythema with a generalized distribution. BP is induced by autoantibodies to structural proteins of the basement membrane zone (BMZ) such as BP180 and BP2301 . Because the majority of BP skin shows complement deposition2 , complement activation is considered to be important for blister formation. Among IgG subclasses, IgG1 have a high ability to activate complements, whereas IgG2 and IgG4 have a low and no ability, respectively...
March 6, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Ken Kamata, Tomohiro Watanabe, Kosuke Minaga, Warren Strober, Masatoshi Kudo
Autoimmune pancreatitis (AIP) is a chronic fibro-inflammatory disorder of the pancreas. However, extensive clinico-pathological analyses have revealed that AIP is, in reality, a pancreatic manifestation of a newly described systemic disease known as IgG4-related disease (IgG4-RD). IgG4-RD is characterized by enhanced local and systemic IgG4 antibody (Ab) responses as well as inflammation involving multiple organs, including the pancreas, bile ducts, and salivary glands. Although mice lack the IgG4 Ab subtype, autoimmune-prone MRL/Mp mice treated with repeated injection with polyinosinic-polycytidylic acid (poly (I:C)) provide an experimental model of AIP...
February 21, 2018: Current Protocols in Immunology
Nissreen Mohammad, Vishal Avinashi, Edmond Chan, Bruce A Vallance, Elodie Portales-Casamar, Jonathan W Bush
OBJECTIVE: Eosinophilic esophagitis (EoE) is considered a TH2-mediated food allergy disease that leads to submucosal esophageal fibrosis and strictures. Recent studies focused on adults with EoE identified a strong association with elevated esophageal IgG4 immunostaining. Our study aimed to determine the association of IgG4 with EoE in pediatric patients. METHODS: Using our local EoE research registry, we identified 41 adequate biopsies from EoE patients. We used 10 age- and sex-matched patients with no diagnostic abnormalities at endoscopy or on biopsy...
March 5, 2018: Journal of Pediatric Gastroenterology and Nutrition
Camila Cortés, Silvia Bielsa, Josep Manel Fernandez, Felip Vilardell Villellas, Blanca Gonzalez-Farré, Josep Manel Casanova
No abstract text is available yet for this article.
March 2, 2018: Acta Dermato-venereologica
Mohammed A K Mahdy, Rashad Abdul-Ghani, Thaker A A Abdulrahman, Samira M A Al-Eryani, Abdulsalam M Al-Mekhlafi, Sami A A Alhaidari, Ahmed A Azazy
BACKGROUND: Onchocerciasis in Yemen is one of the most neglected diseases, where baseline estimates of onchocerciasis and monitoring of the impact of ivermectin regularly administered to the affected individuals on its transmission are lacking. Therefore, this study aimed to determine the anti-Ov16 IgG4 seroprevalence among local communities of Hodeidah and Al-Mahwit governorates of Tihama Region. The factors possibly associated with previous exposure to infection were also studied. METHODOLOGY/PRINCIPAL FINDINGS: This cross-sectional study was conducted in two ivermectin-targeted districts endemic for onchocerciasis in Hodeidah and Al-Mahwit and two untargeted districts with unknown previous endemicity in Hodeidah between February and July 2017...
March 5, 2018: PLoS Neglected Tropical Diseases
Robby Engelmann, Sven Nekarda, Daniela Kuthning, Christian Kneitz, Brigitte Müller-Hilke
The treatment options for patients suffering from rheumatoid arthritis expanded over the last years. However, reliable biomarkers to guide therapy decisions are still warranted. Therefore, we here evaluated the value of antibodies against citrullinated peptide antigens (ACPA) IgG subclasses and peripheral blood antigen presenting cells as biomarkers to monitor and predict therapy response of patients with rheumatoid arthritis. Thirty-four ACPA-positive RA patients were enrolled and monitored for 3 months after therapy begin...
March 3, 2018: Clinical Rheumatology
Jie Chang, Wen Zhang
IgG4-related disease (IgG4-RD) is a chronic and systemic disease that can involve multiple organs. The most commonly involved organs include the salivary glands, orbital adnexal structures, paranasal sinus, thyroid, lungs, breasts, aorta, pancreas, biliary ducts, kidneys, retroperitoneum, lymph nodes, prostate, pituitary, and endocranium. Due to increased disease research, several new site-specific nuances of IgG4-RD have been described. The authors have reviewed the recent literature and briefly summarize the infrequent organ involvement of IgG4-RD...
March 3, 2018: Clinical Rheumatology
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