Review
Immunology
Jiachen Liu, Wei Yin, Lisa S. Westerberg, Pamela Lee, Quan Gong, Yan Chen, Lingli Dong, Chaohong Liu
Summary: IgG(4)-RD is a newly discovered autoimmune disease characterized by elevated serum IgG(4) concentrations and multi-organ fibrosis. The pathogenesis of this disease involves abnormal adaptive immune cells and cytokines, including different types of T and B cells, and cytokines secreted by these cells, leading to fibrosis in multiple organs.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Medical Laboratory Technology
Keiichi Nakano, Junichi Sugita, Masanori Seimiya, Keiko Yasuda, Chiaki Watanabe, Takanori Teshima
Summary: This study found that the formation frequency of IgG4-IgE complex was significantly higher in patients with IgG4-RD compared to those without IgG4-RD. There were no significant differences in clinical characteristics and laboratory data between the groups, but the IgG4-IgE complex-positive group had a significantly higher frequency of pancreatic lesions and a significantly lower rate of retroperitoneal fiber/periarterial lesions compared to the IgG4-IgE complex-negative group.
CLINICA CHIMICA ACTA
(2022)
Article
Medical Laboratory Technology
Keiichi Nakano, Junichi Sugita, Masanori Seimiya, Keiko Yasuda, Chiaki Watanabe, Takanori Teshima
Summary: This study reported a case of IgG4-IgE complex in a patient with IgG4-RD, leading to discrepancies in IgE concentrations among measuring reagents.
CLINICA CHIMICA ACTA
(2022)
Review
Respiratory System
Romain Muller, Mikael Ebbo, Paul Habert, Laurent Daniel, Antoine Briantais, Pascal Chanez, Jean Yves Gaubert, Nicolas Schleinitz
Summary: IgG4-related disease is a rare systemic fibroinflammatory disease that can affect various organs, including the thoracic region. Diagnosing thoracic involvement requires a comprehensive approach, including clinical, biological, imaging, and histopathological evaluations. Steroids are recommended as the first-line treatment for this disease.
Review
Immunology
Siyu Wu, Haiqiang Wang
Summary: IgG4-related digestive diseases are a group of chronic inflammatory disorders that are often misdiagnosed as malignancies. A comprehensive understanding of these diseases remains challenging, and current diagnosis relies on integrated diagnostic criteria and specific organ involvement criteria. Further research is needed to elucidate the underlying mechanisms and explore potential treatments.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Rheumatology
Yanying Liu, Lijuan Zhu, Zhenfan Wang, Qiaozhu Zeng, Fei Yang, Jingyuan Gao, Ziqiao Wang, Kunkun Wang, Limin Ren, Yanyan Zhang, Shanshan Zhang, Yi Wang, Danhua Shen, Changsheng Xia, Guangyan Yu, Zhanguo Li
Summary: This study compared demographic, clinical, and laboratory characteristics of IgG4-related disease patients with and without retroperitoneum involvement. The results revealed significant differences in age, gender, organ involvement, and laboratory parameters between the two groups.
Article
Medicine, General & Internal
Linyang Gan, Xuan Luo, Yunyun Fei, Linyi Peng, Jiaxin Zhou, Jieqiong Li, Hui Lu, Zheng Liu, Panpan Zhang, Xiaowei Liu, Wen Zhang
Summary: Relapse was common in patients with IgG4-ROD, with those receiving combination therapy showing a lower relapse rate and shorter glucocorticoid therapy duration. Presence of multiple ocular lesions was associated with a higher risk of relapse, and achieving complete response at the 6th month may predict a better prognosis in IgG4-ROD.
FRONTIERS IN MEDICINE
(2021)
Review
Biochemistry & Molecular Biology
Mara Carsote, Claudiu Nistor
Summary: Recently, Riedel's thyroiditis has been classified as part of the larger spectrum of IgG4-related disease. This study aimed to review the latest data on Riedel's thyroiditis, particularly focusing on IgG4-related disease and IgG4-related thyroid disease. The analysis included a comprehensive review of articles and selected original studies, highlighting the importance of early suspicion for accurate diagnosis and prompt treatment.
Article
Pharmacology & Pharmacy
Riccardo Capecchi, Cristina Croia, Ilaria Puxeddu, Federico Pratesi, Andrea Cacciato, Daniela Campani, Ugo Boggi, Luca Morelli, Antonio Tavoni, Paola Migliorini
Summary: The study revealed significantly higher levels of serum SDF-1/CXCL12 in IgG4-RD patients compared to normal controls, suggesting a potential role of this chemokine in the pathogenesis of IgG4-RD. Additionally, the IgG4-RD AIP subgroup showed higher serum levels of SDF-1/CXCL12 compared to the SSj group.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Health Care Sciences & Services
Wei-Yi Chou, Ching-Yao Tsai, Chieh-Chih Tsai
Summary: This study analyzed the association between long-term changes in serum IgG4 levels and the clinical course of IgG4-related ophthalmic disease (IgG4-ROD) patients. The study found that patients who had a greater response to initial steroid therapy were more likely to have normalized IgG4 levels in the long term. Some patients remained in remission despite persistently elevated IgG4 levels.
JOURNAL OF PERSONALIZED MEDICINE
(2022)
Editorial Material
Medicine, General & Internal
Stephanie V. Sherman, Yi-Min Huang, Pei-Hsuan Lin
Summary: A 67-year-old man presented with swollen eyelids and proptosis in the ophthalmology clinic. Biopsy of the right lacrimal gland revealed a lymphoplasmacytic infiltrate with IgG4-positive plasma cells.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Article
Genetics & Heredity
Takeji Umemura, Yasunari Fujinaga, Norihiro Ashihara, Makiko Ozawa, Yasuhiro Kuraishi, Takayuki Watanabe, Hideaki Hamano, Akira Meguro, Shigeyuki Kawa, Masao Ota
Summary: The study revealed that genetic variations in the IL1R1 gene are associated with the development of IgG4-related periaortitis/periarteritis, with specific SNPs and haplotypes showing significant correlations with the disease manifestation in patients.
Article
Immunology
Oh Chan Kwon, Min-Chan Park, Yong-Gil Kim
Summary: The study aimed to identify serologic parameters associated with the disease activity of IgG4-related disease. In patients with normal serum IgG4 concentrations, only IgG concentration was correlated with disease activity. However, in patients with elevated serum IgG4 concentrations, ESR, C3, C4, IgG4 concentration, IgG concentration, and IgG4/IgG ratio were correlated with disease activity. Among these parameters, the combination of C3 and IgG4 concentration had the strongest correlation coefficient in this group.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Health Care Sciences & Services
Magda Baranska, Joanna Makowska, Malgorzata Wagrowska-Danilewicz, Wioletta Pietruszewska
Summary: This paper aims to analyze the different aspects of IgG4-related disease (IgG4-RD) presenting as supraglottic stenosis, and compare the usefulness of different criteria and discuss treatment options. By analyzing nine papers and a case study, we found that when treating IgG4-RD, clinicians should suspect autoimmune etiology and implement specific treatment.
JOURNAL OF PERSONALIZED MEDICINE
(2022)
Article
Medicine, General & Internal
Kensuke Namba, Daiki Sakai, Hiroshi Mikamo, Yuta Sugizaki, Yoshiya Sugiura, Nobuyuki Hiruta, Yasuo Matsuzawa, Kaichi Kaneko
Summary: We report a rare case of a cardiac mass in a patient with IgG4-related disease (IgG4-RD) and discuss 10 similar cases previously reported. The patient was diagnosed with IgG4-RD and underwent external pacemaker implantation and steroid therapy. This case suggests that combination therapy may be effective in patients with IgG4-RD and cardiac mass, potentially avoiding the need for invasive treatments such as surgery.
Review
Immunology
Marco Lanzillotta, Andreu Fernandez-Codina, Emma Culver, Mikael Ebbo, Fernando Martinez-Valle, Nicolas Schleinitz, Emanuel Della-Torre
Summary: Awareness of IgG4-related disease (IgG4-RD) is increasing globally, with glucocorticoids being the primary treatment, but the need for repeated courses to maintain remission. Without treatment, it can lead to organ dysfunction and death, making the identification of new therapeutic targets crucial. Targeting B and T-lymphocyte activation may hold promise as an alternative treatment strategy.
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
(2021)
Article
Gastroenterology & Hepatology
Kit Curtius, Misha Kabir, Ibrahim Al Bakir, Chang Ho Ryan Choi, Juanda L. Hartono, Michael Johnson, James E. East, James O. Lindsay, Roser Vega, Siwan Thomas-Gibson, Janindra Warusavitarne, Ana Wilson, Trevor A. Graham, Ailsa Hart
Summary: This study identified that patients with large, unresected, multifocal LGD and recent moderate/severe inflammation are at highest risk of developing advanced neoplasia. Personalised risk prediction provided via the Ulcerative Colitis-Cancer Risk Estimator can support treatment decision-making.
Article
Gastroenterology & Hepatology
Daniel E. Radford-Smith, Emmanuel A. Selvaraj, Rory Peters, Michael Orrell, Jonathan Bolon, Daniel C. Anthony, Michael Pavlides, Kate Lynch, Alessandra Geremia, Adam Bailey, Emma L. Culver, Fay Probert
Summary: Serum metabolomic profiling has the potential to accurately distinguish between PSC and IgG4-SC, improve the diagnosis of IgG4-RD, and differentiate IgG4-SC from PSC.
LIVER INTERNATIONAL
(2022)
Letter
Rheumatology
Giuseppe A. Ramirez, Marco Lanzillotta, Mikael Ebbo, Andreu Fernandez-Codina, Gaia Mancuso, Olimpia Orozco-Galvez, Lorenzo Dagna, Nicolas Schleinitz, Fernando Martinez-Valle, Emma L. Culver, Emanuel Della-Torre
Article
Gastroenterology & Hepatology
Charlotte Lloyd, Jessica Leighton, Lin Lee Wong, Anna Goulding, Ann Brownlee, Penney Gray, Emma Culver, Neil Halliday, Doug Thorburn, Michael A. Heneghan, David E. J. Jones, Catherine Exley, Jessica K. Dyson
Summary: This study explored the experiences and needs of patients with autoimmune hepatitis. The findings revealed that patients require more support networks and empowerment, while also facing issues of stigma and fatigue. Patient priorities for improving care include slowing disease progression, avoiding corticosteroids, and minimizing side effects. The study also found that patients are willing to participate in clinical trials.
DIGESTIVE DISEASES AND SCIENCES
(2023)
Article
Gastroenterology & Hepatology
Nadir Abbas, Emma L. Culver, Douglas Thorburn, Neil Halliday, Hannah Crothers, Jessica K. Dyson, April Phaw, Richard Aspinall, Salim I. Khakoo, Yiannis Kallis, Belinda Smith, Imran Patanwala, Anne McCune, Chenchu R. Chimakurthi, Vinod Hegade, Michael Orrell, Rebecca Jones, George Mells, Colette Thain, Robert-Mitchell Thain, David Jones, Gideon Hirschfield, Palak J. Trivedi
Summary: This study aimed to evaluate the effectiveness of obeticholic acid (OCA) and fibric acid derivatives as second-line treatments for primary biliary cholangitis. The study found that rates of biochemical response and drug discontinuation were similar between fibric acid derivatives and OCA in patients in the United Kingdom.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2023)
Article
Medicine, General & Internal
Mamta Bajre, Mina Moawad, Elizabeth Shumbayawonda, Jane Elizabeth Carolan, Julie Hart, Emma Culver, Michael Heneghan
Summary: This study aimed to conduct early economic modeling to assess the affordability of using mpMR as an alternative to liver biopsy. The results showed that the minimum cost savings of using mpMR instead of biopsy for moderate/severe AIH patients were 232,333 pounds per year. For mild/moderate AIH patients, three mpMR scans over 5 years could save an estimated minimum cost of 139,400 pounds.
Letter
Gastroenterology & Hepatology
Ida Schregel, Guilherme P. Ramos, Stephanie Ioannou, Emma Culver, Martti Farkkila, Christoph Schramm
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2023)
Review
Gastroenterology & Hepatology
Kathleen Moeller, Barbara Braden, Emma L. Culver, Christian Jenssen, Ehsan Safai Zadeh, Amjad Alhyari, Christian Goerg, Andre Ignee, Michael Hocke, Yi Dong, Siyu Sun, Siegbert Faiss, Christoph F. Dietrich
Summary: Sclerosing cholangitis is a chronic progressive cholestatic disease characterized by inflammation, fibrosis, and stricturing of the biliary system. It is important to differentiate between primary and secondary sclerosing cholangitis due to differences in treatment, risk of malignancy, and progression to complications. This review focuses on secondary sclerosing cholangitis and discusses the pathogenic mechanisms, risk factors, clinical presentation, and imaging modalities for distinguishing between these conditions.
ENDOSCOPIC ULTRASOUND
(2023)
Article
Gastroenterology & Hepatology
Piotr Eder, Bram Verstock, Emma Culver, Gabriele Dragoni, Lea Isabell Kredel, Joanna Wypych, Ana Garcia Garcia de Paredes, Magdalena Kaniewska, Haim Leibovitzh, Triana Lobaton, Marie Truyens, Grzegorz Oracz, Davide Giuseppe Ribaldone, Teresa Starzynska, Abdenor Badaoui, Jean-Francois Rahier, Cristina Bezzio, Peter Bossuyt, Katherine Falloon, Daniela Pugliese, Catherine Frakes Vozzo, Tine Jess, Lone Larsen, Soren Schou Olensen, Partha Pal, Maria Chaparro, Dikla Dror, Pierre Ellul, Iga Gromny, Maria Janiak, Katarzyna Maciejewska, Noam Peleg, Ariella Bar-Gil Shitrit, Lukasz Szwed, Renata Talar-Wojnarowska, Yifat Snir, Roni Weisshof, Eran Zittan, Izabela Miechowicz, Idan Goren
Summary: This study investigated the long-term outcomes of autoimmune pancreatitis (AIP) and inflammatory bowel disease (IBD) in patients with coexisting AIP-IBD, as well as the predictors of complicated AIP course. The majority of patients had type 2 AIP and colonic IBD, and the AIP course was relatively benign with favorable prognosis, but a quarter of the patients developed pancreatic complications. Age, family history, and CD were identified as potential predictors of uncomplicated AIP course.
JOURNAL OF CROHNS & COLITIS
(2023)
Article
Medicine, General & Internal
Weiqi Liao, Carol A. C. Coupland, Hamish Innes, Peter Jepsen, Philippa C. Matthews, Cori Campbell, The DeLIVER consortium DeLIVER consortium, Eleanor Barnes, Julia Hippisley-Cox
Summary: This study aims to understand the disparities in epidemiology and clinical pathways of primary liver cancer in England. The findings show that age, sex, socioeconomic deprivation, ethnicity, and geographical regions are all significantly associated with liver cancer incidence. Patients aged 80 years and above have a higher risk of being diagnosed at late stages and have poorer survival rates. Men are at a higher risk of liver cancer than women, and survival rates are generally poor.
Letter
Rheumatology
Rory James Redmond Peters, Harry Martin, Aleena Virdee, Eve Fryer, Helen Bungay, Manuel Rodriguez-Justo, Manil Chouhan, Eleanor Barnes, George Webster, Emma L. Culver
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Medicine, General & Internal
Michael A. Heneghan, Elizabeth Shumbayawonda, Andrea Dennis, Refah Z. Ahmed, Mussarat N. Rahim, Michael Ney, Loren Smith, Matt Kelly, Rajarshi Banerjee, Emma L. Culver
Summary: This study investigates the utility of multiparametric MRI in patients with autoimmune hepatitis (AIH) and demonstrates its significant impact on clinical management and patient risk stratification.
Review
Rheumatology
L. Iaccarino, R. Talarico, E. Bozzalla-Cassione, G. R. Burmester, E. L. Culver, A. Doria, M. Ebbo, P. M. van Hagen, E. Hachulla, J. A. M. van Laar, M. Lanzillotta, F. Martinez-Valle, C. Montecucco, S. Monti, C. Nalli, N. Schleinitz, A. Tincani, E. Della-Torre, T. Alexander
Summary: Immunoglobulin G4 (IgG4)-related disease is a chronic fibroinflammatory condition characterized by an accumulation of IgG4-secreting plasma cells. Diagnosing this disease is challenging, but biomarkers can be used for assisting diagnosis and monitoring.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2022)
Article
Gastroenterology & Hepatology
Emmanuel A. Selvaraj, Ahmed Ba-Ssalamah, Sarah Poetter-Lang, Gerard R. Ridgway, J. Michael Brady, Jane Collier, Emma L. Culver, Adam Bailey, Michael Pavlides
Summary: Quantitative biliary analysis using MRCP+ was effective in distinguishing abnormal from normal bile ducts in patients with PSC. Intrahepatic biliary dilatation severity calculated using MRCP+ was elevated in high-risk PSC patients and may be used for risk stratification. This exploratory study laid the groundwork for further investigation of novel quantitative biliary metrics in multicenter studies.
HEPATOLOGY COMMUNICATIONS
(2022)