keyword
https://read.qxmd.com/read/38547341/igg4-related-disease-complicated-with-diffuse-and-chronic-gastrointestinal-inflammation-leading-to-small-intestinal-perforation
#1
JOURNAL ARTICLE
Kazuma Ino, Yoshiyuki Arinuma, Masashi Akiya, Sabine Kajita, Sosei Okina, Junichi Sakamoto, Tomoki Tanaka, Yu Matsueda, Tatsuhiko Wada, Sumiaki Tanaka, Kenji Oku, Kunihiro Yamaoka
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by elevated serum IgG4, IgG4+ cell infiltration, storiform fibrosis, and obliterative phlebitis. While IgG4-RD can affect various organs, gastrointestinal tract involvement is less common. Here, we report a 70-year-old female with IgG4-RD complicated with diffuse and chronic gastrointestinal inflammation which led to small intestinal perforation. She had been suffering from anorexia, abdominal pain, vomiting, and diarrhea, and hospitalized due to recurrent ileus...
March 28, 2024: Modern rheumatology case reports
https://read.qxmd.com/read/38541223/overlapping-case-of-advanced-systemic-sclerosis-and-igg4-related-disease-after-autologous-hematopoietic-stem-cell-transplantation
#2
Alisa Julija Dulko, Irena Butrimiene, Alma Cypiene, Valdas Peceliunas, Donatas Petroska, Ernesta Stankeviciene, Rita Rugiene
Both scleroderma and immunoglobulin G4-related disease (IgG4-RD) are systemic fibro-inflammatory diseases characterised by lymphoplasmacytic infiltrates. IgG4-RD and systemic sclerosis (SSc) may share common pathophysiological mechanisms, but no examples of co-occurrence of the diseases have been found. Autologous haematopoietic stem cell transplantation (AHSCT) is implemented in selected rapidly progressive SSc with a high risk of organ failure. However, existing guidelines are based on clinical trials that do not represent the entire patient population and exclude critically ill patients with no therapeutic alternatives...
March 18, 2024: Medicina
https://read.qxmd.com/read/38537149/urinary-bladder-involvement-in-igg4-related-disease-a-case-based-review
#3
JOURNAL ARTICLE
Parag Vijayvergia, Sayan Mukherjee, Lily Singh, Urmila Dhakad
Immunoglobulin G4-related disease (IgG4-RD) is an immune-driven fibroinflammatory disease that presents as tumefactive lesions that not only commonly affects the pancreas, lacrimal and salivary glands, lung, liver and kidney but can also affect any organs. However, involvement of the urinary bladder in IgG4-RD is rarely reported. We describe a case of IgG4-RD involving the urinary bladder mimicking carcinoma and review the published literature-a 39-year-old male presented with complaints of dysuria, urgency and hesitancy...
March 2, 2024: Modern rheumatology case reports
https://read.qxmd.com/read/38530663/effectiveness-of-tofacitinib-monotherapy-for-patients-with-igg4-rd-or-idiopathic-retroperitoneal-fibrosis
#4
JOURNAL ARTICLE
Xiaoyu Cao, Shaoxiang Li, Jin Wan, Zhibo Yu, Gehong Dong, Wei Zhou
OBJECTIVES: To explore the effectiveness of tofacitinib for immunoglobulin G4-related disease (IgG4-RD) and idiopathic retroperitoneal fibrosis (IRF), and investigate the expression of JAKs in the lesion of these diseases. METHODS: Clinical data of patients with IgG4-RD or IRF who were administered with tofacitinib monotherapy were collected. IgG4-RD responder index (IgG4-RD RI) was assessed. The expression of JAK1, JAK2, JAK3, and TYK2 were analysed with immunohistochemistry staining in three salivary glands specimens of IgG4-RD and one retroperitoneal tissue of IRF...
March 26, 2024: Clinical and Experimental Rheumatology
https://read.qxmd.com/read/38530483/radiology-domain-in-the-diagnosis-of-igg4-rd-according-to-the-2019-american-college-of-rheumatology-and-european-league-against-rheumatism-classification
#5
JOURNAL ARTICLE
Khaled Y Elbanna, Jie-Ying Kowa, Nikhil Mirajkar, Korosh Khalili, Tae Kyoung Kim
OBJECTIVES: To evaluate the performance of radiology-related inclusion criteria of the 2019 ACR-EULAR classification system in the diagnosis of IgG4-related disease (IgG4-RD). METHODS: This retrospective single-institution study included patients who received a diagnosis of IgG4-RD between January 2010 and December 2020. Two abdominal radiologists independently reviewed baseline imaging studies and scored radiology findings according to the 2019 ACR-EULAR classification criteria...
March 26, 2024: Insights Into Imaging
https://read.qxmd.com/read/38524667/laminin-511-e8-an-autoantigen-in-igg4-related-cholangitis-contributes-to-cholangiocyte-protection
#6
JOURNAL ARTICLE
David C Trampert, Remco Kersten, Dagmar Tolenaars, Aldo Jongejan, Stan F J van de Graaf, Ulrich Beuers
BACKGROUND & AIMS: IgG4-related cholangitis (IRC) is the hepatobiliary manifestation of IgG4-related disease. Anti-laminin 511-E8 autoantibodies have been identified in its pancreatic manifestation. Laminin 511-E8 promotes endothelial barrier function, lymphocyte recruitment, and cholangiocyte differentiation. Here, we investigate anti-laminin 511-E8 autoantibody presence in IRC, and mechanisms via which laminin 511 may contribute to cholangiocyte protection. METHODS: Anti-laminin 511-E8 serum autoantibody positivity was assessed by ELISA...
April 2024: JHEP reports: innovation in hepatology
https://read.qxmd.com/read/38523581/adult-ocular-adnexal-xanthogranulomatous-disease-associated-with-immunoglobulin-g4-related-disease-an-unusual-association
#7
JOURNAL ARTICLE
A R De Santana, M D L Castro de Oliveira Figueirôa, A L Souza Pedreira, G L Behrens Pinto, M B Santiago
Adult-onset xanthogranuloma (AOX) and immunoglobulin G4-related disease (IgG4-RD) are uncommon fibrosing conditions that may exhibit localized ocular manifestations and occasionally systemic symptoms. These conditions exhibit overlapping clinical and histological features, suggesting a potential correlation between them, although their exact relationship remains unclear. This paper presents the case of a black male patient exhibiting typical histological indications of both AOX and IgG4-RD. The patient responded positively to corticosteroid treatment...
March 22, 2024: Reumatismo
https://read.qxmd.com/read/38521787/expression-of-usp25-associates-with-fibrosis-inflammation-and-metabolism-changes-in-igg4-related-disease
#8
JOURNAL ARTICLE
Panpan Jiang, Yukai Jing, Siyu Zhao, Caini Lan, Lu Yang, Xin Dai, Li Luo, Shaozhe Cai, Yingzi Zhu, Heather Miller, Juan Lai, Xin Zhang, Xiaochao Zhao, Yonggui Wu, Jingzhi Yang, Wen Zhang, Fei Guan, Bo Zhong, Hisanori Umehara, Jiahui Lei, Lingli Dong, Chaohong Liu
IgG4-related disease (IgG4-RD) has complex clinical manifestations ranging from fibrosis and inflammation to deregulated metabolism. The molecular mechanisms underpinning these phenotypes are unclear. In this study, by using IgG4-RD patient peripheral blood mononuclear cells (PBMCs), IgG4-RD cell lines and Usp25 knockout mice, we show that ubiquitin-specific protease 25 (USP25) engages in multiple pathways to regulate fibrotic and inflammatory pathways that are characteristic to IgG4-RD. Reduced USP25 expression in IgG4-RD leads to increased SMAD3 activation, which contributes to fibrosis and induces inflammation through the IL-1β inflammatory axis...
March 23, 2024: Nature Communications
https://read.qxmd.com/read/38496223/a-case-of-isolated-central-nervous-system-rosai-dorfman-disease
#9
Fatma Ebru Algül, Begüm Yeni Erdem, Gülçin Yeğen, Servet Yolbaş
Rosai-Dorfman disease (RDD) is a benign histiocytosis with unknown etiology. It generally occurs in cervical lymph nodes. Isolated central nervous system (CNS) RDD is very rare in the literature. We reported a case of no systemic involvement Rosai-Dorfmann which is rarely seen and shows CNS involvement by mimicking meningioma. A 32-year-old man presented with diplopia and a headache he has been experiencing for the past two years. His neurological examination showed left facial paresthesia, consistent with trigeminal nerve trace...
2024: Noro Psikiyatri Arsivi
https://read.qxmd.com/read/38494710/validation-of-the-diagnostic-criteria-for-igg4-related-periaortitis-periarteritis-and-retroperitoneal-fibrosis-igg4pa-rpf-2018-and-proposal-of-a-revised-2023-version-for-igg4-related-cardiovascular-retroperitoneal-disease
#10
JOURNAL ARTICLE
Ichiro Mizushima, Noriyasu Morikage, Eisaku Ito, Fuminori Kasashima, Yasushi Matsumoto, Naoki Sawa, Hajime Yoshifuji, Takako Saeki, Yukako Shintani-Domoto, Shogo Shimada, Toshio Takayama, Eisuke Amiya, Makiko Ozawa, Masaaki Takahashi, Yasunari Fujinaga, Takahiro Katsumata, Yukio Obitsu, Atsushi Izawa, Hiroyuki Kanno, Noriko Oyama-Manabe, Nobukazu Ishizaka, Tasuku Nagasawa, Hiroki Takahashi, Takao Ohki, Mitsuhiro Kawano, Satomi Kasashima
BACKGROUND: In 2018, diagnostic criteria were introduced for IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis (PA/RPF). This study assessed the existing criteria and formulated an improved version.Methods and Results: Between August 2022 and January 2023, we retrospectively analyzed 110 Japanese patients diagnosed with IgG4-related disease (IgG4-RD) involving cardiovascular and/or retroperitoneal manifestations, along with 73 non-IgG4-RD patients ("mimickers") identified by experts...
March 16, 2024: Circulation Journal: Official Journal of the Japanese Circulation Society
https://read.qxmd.com/read/38472948/a-case-of-castleman-s-disease-with-a-marked-infiltration-of-igg4-positive-cells-in-the-renal-interstitium
#11
Erika Sawada, Yuya Shioda, Kohki Ogawa, Takatsugu Iwashita, Yuko Ono, Hajime Hasegawa, Akito Maeshima
Multicentric Castleman's disease (MCD) is a benign lymphoproliferative disorder with heterogenous clinical symptoms, and involves systemic organs in addition to lymph nodes. Herein, we present the case of a 55-year-old man with MCD characterized by an extensive infiltration of IgG4+ plasma cells in the kidneys. The patient presented to our hospital with a high fever and diarrhea. On admission, laboratory analysis revealed anemia, renal dysfunction (eGFR 30 mL/min/1.73 m2 ), polyclonal gammopathy (IgG 7130 mg/dL), elevated serum IgG4 level (2130 mg/dL), and increased C-reactive protein (8...
February 23, 2024: Diagnostics
https://read.qxmd.com/read/38458761/the-development-and-initial-validation-of-igg4-related-disease-damage-index-a-consensus-report-from-chinese-igg4-rd-consortium
#12
JOURNAL ARTICLE
Linyi Peng, Jingna Li, Jiaxin Zhou, Yunyun Fei, Lingli Dong, Yan-Ying Liu, Dingding Zhang, Yanhong Wang, Shuhong Chi, Fang Wang, Yunxia Hou, Xiaoping Hong, Hongsheng Sun, Yujin Ye, Nan Che, Rong Zhang, Changyan Liu, Zongfei Ji, Wenjia Sun, Cheng Zhao, Ning Ma, Yamin Lai, Mengtao Li, Yan Zhao, Xiaofeng Zeng, Liwei Lu, Wen Zhang
OBJECTIVE: To develop and conduct an initial validation of the Damage Index for IgG4-related disease (IgG4-RD DI). METHODS: A draft of index items for assessing organ damages in patients with IgG4-RD was generated by experts from the Chinese IgG4-RD Consortium (CIC). The preliminary DI was refined using the Delphi method, and a final version was generated by consensus. 40 IgG4-RD cases representing four types of clinical scenarios were then selected, each with two time points of assessment for at least 3 years of follow-up...
March 8, 2024: RMD Open
https://read.qxmd.com/read/38455056/a-case-of-igg4-related-ophthalmic-disease-after-sars-cov-2-vaccination-case-report-and-literature-review
#13
REVIEW
Peixuan Zhang, Qian Wu, Xiao Xu, Minliang Chen
Coronavirus disease 19 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is affecting the world with a surge in cases. A variety of autoimmune diseases occur after SARS-CoV-2 infection or vaccination, of which IgG4-related disease (IgG4-RD) is an important type. IgG4-RD can involve multiple organs of the body. The ocular manifestation of IgG4-RD is called IgG4-related ophthalmic disease (IgG4-ROD). We herein report a patient diagnosed with IgG4-ROD. The patient developed ptosis and vision loss after SARS-CoV-2 vaccination, and the symptoms worsened after SARS-CoV-2 infection...
2024: Frontiers in Immunology
https://read.qxmd.com/read/38443012/igg4-related-neurological-disease-a-single-center-ambispective-study-from-south-india
#14
JOURNAL ARTICLE
Y Muralidhar Reddy, Subhendu Parida, Lalitha Pidaparthi, Shyam Kumar Jaiswal, Vijaya Tourani, Syed Osman, B Santosh Kumar, Jagarlapudi M K Murthy
IgG4-related disease (IgG4-RD) is an immune-mediated multi-system disorder. The nervous system (IgG4-RND) is rarely affected. We describe a short case series. We performed an ambispective analysis of IgG4-RND patients admitted at our centre between January 2016 and December 2022. Eight patients (M: F-2:6) were included with a mean age at presentation of 40.63 ± 17.88 years and disease duration of 5.16 ± 4.08 years. The common diseased sites were pachymeninges (7), orbits (4), paranasal sinuses (3), frontal lobe (1), hypophysis (1), leptomeninges (1), and middle ear (1)...
January 1, 2024: Neurology India
https://read.qxmd.com/read/38433835/igg4-autoantibodies-and-autoantigens-in-the-context-of-igg4-autoimmune-disease-and-igg4-related-disease
#15
REVIEW
Rodrigo V Motta, Emma L Culver
Immunoglobulins are an essential part of the humoral immune response. IgG4 antibodies are the least prevalent subclass and have unique structural and functional properties. In this review, we discuss IgG4 class switch and B cell production. We review the importance of IgG4 antibodies in the context of allergic responses, helminth infections and malignancy. We discuss their anti-inflammatory and tolerogenic effects in allergen-specific immunotherapy, and ability to evade the immune system in parasitic infection and tumour cells...
2024: Frontiers in Immunology
https://read.qxmd.com/read/38426159/myelodysplastic-syndrome-with-igg4%C3%A2-related-disease-a-case-report
#16
Lina Wang, Xi Pang, Zirui Hong, Baodong Ye, Zhiping Hu, Chunli Zhang, Shuyan Liu
At present, to the best of our knowledge, there are only a few case reports of IgG4-related disease (IgG4-RD) involving myelodysplastic syndrome (MDS), yet the incidence of MDS and IgG4-RD is increasing in middle-aged and elderly people. The present study presents a case of MDS combined with IgG4-RD admitted to Zhejiang Provincial Hospital of Chinese Medicine in September 2022. The (66-year-old; male) patient was admitted to the hospital due to hematopenia with an elevated IgG4 index. The diagnosis of MDS combined with IgG4-RD was confirmed after various exams, including pathological examination...
April 2024: Oncology Letters
https://read.qxmd.com/read/38395839/pla2r-positive-membranous-nephropathy-in-igg4-related-disease
#17
JOURNAL ARTICLE
Yusuke Ushio, Taro Akihisa, Kazunori Karasawa, Momoko Seki, Shizuka Kobayashi, Yoei Miyabe, Hiroshi Kataoka, Naoko Ito, Sekiko Taneda, Shin'ichi Akiyama, Akira Hebisawa, Mitsuhiro Kawano, Kazuho Honda, Junichi Hoshino
BACKGROUND: IgG4-related disease (IgG4-RD) is a fibroinflammatory disease that affects multiple organs, including the pancreas, lacrimal glands, salivary glands, periaortic/retroperitoneum, and kidney. Interstitial nephritis is a typical renal disorder associated with IgG4-RD, but membranous nephropathy is also seen in some cases. CASE PRESENTATION: Herein we report on the case of a 77-year-old male patient with nephrotic syndrome and IgG4-related lung disease. His serum phospholipase A2 receptor (PLA2R) antibody was positive...
February 23, 2024: BMC Nephrology
https://read.qxmd.com/read/38390429/a-case-report-of-immunoglobulin-g4-related-hypertrophic-sclerosing-pachymeningitis
#18
Yan Zhang, Huijuan Chen, Feng Chen
IgG4-related diseases (IgG4-RD) are a group of chronic progressive autoimmune diseases of unknown etiology that are increasingly recognized as an important pathophysiological basis for a variety of systemic diseases. It is thought to involve almost any organ of the body, but the involvement of the central nervous system is relatively rare. We report the case of a 56-year-old male patient admitted to the hospital d recurrent dizziness and nausea for more than 3 months. The preoperative imaging was misdiagnosed as a meningioma, with this lesion demonstrated localized inhomogeneous thickening of the meninges in the left parietal region on T2-weighted and T2 fat suppression sequences with localized nodular changes...
May 2024: Radiology Case Reports
https://read.qxmd.com/read/38389040/semiquantitative-18-f-fdg-pet-ct-in-monitoring-glucocorticoid-response-of-immunoglobulin-g4-related-effusive-constrictive-pericarditis-a-case-report
#19
JOURNAL ARTICLE
Soo Yeon An, Byung Joo Sun
BACKGROUND: Immunoglobulin G4 (IgG4)-related effusive constrictive pericarditis (ECP) is a rare manifestation of IgG4-related disease (IgG4-RD). It can lead to persistent pericardial fibrosis, resulting in cardiac tamponade, diastolic dysfunction, and heart failure. Glucocorticoids are the primary treatment for effectively reducing inflammation and preventing fibrosis. However, guidelines for monitoring treatment response are lacking and tapering glucocorticoid therapy for specific target organs remains a challenge...
February 22, 2024: BMC Cardiovascular Disorders
https://read.qxmd.com/read/38381285/immunohistological-analysis-reveals-igg1-dominant-immunophenotype-of-tubulointerstitial-nephritis-unassociated-with-igg4-related-diseases
#20
JOURNAL ARTICLE
Toshiki Hyodo, Shigeo Hara, Shunsuke Goto, Hideki Fujii, Shinichi Nishi, Tomoko Horinouchi, Kandai Nozu, Norishige Yoshikawa, Akihiro Yoshimoto, Tomoo Itoh
PURPOSE: Tubulointerstitial nephritis (TIN) has various etiologies, including IgG4-related disease (IgG4-RD), autoimmune diseases, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), and others. IgG4-positive plasma cell infiltration can occasionally be found in TIN unrelated to IgG4-RD. Therefore, there may be problems with usage of IgG4 immunostaining to differentiate between TIN with and TIN without IgG4-RD. This study aimed to compare the proportion of plasma cells that are positive for each IgG subclass and to clarify the predominant IgG subclass trends and clinical characteristics associated with IgG4-RD and non-IgG4-related interstitial nephritis...
February 21, 2024: International Urology and Nephrology
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