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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/29452298/treatment-and-outcomes-in-patients-with-igg4-related-disease-using-the-igg4-responder-index
#1
Andreu Fernández-Codina, Blanca Pinilla, Iago Pinal-Fernández, Cristina López, Guadalupe Fraile-Rodríguez, Eva Fonseca-Aizpuru, Iago Carballo, Pilar Brito-Zerón, Carlos Feijóo-Massó, Miguel López-Dupla, Maria Cinta Cid, Fernando Martínez-Valle
BACKGROUND: IgG4-related disease (IgG4-RD) is an autoimmune disease triggering an inflammatory cascade that leads to fibrosis. Outcome measures are limited and treatment options remain underexplored. OBJECTIVES: To assess the variation of the IgG4 responder index (IgG4-RI) in a cohort of IgG4-RD patients and to explore their treatments and outcomes. METHODS: We studied the clinical phenotype, severity of the disease and response to treatment in an ambispective multicenter cohort study including 14 different hospitals in Spain...
February 13, 2018: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/29434730/clinical-and-pathological-characteristics-of-igg4-related-interstitial-lung-disease
#2
Xiaoting Lv, Feng Gao, Qicai Liu, Sheng Zhang, Zhihua Huang, Yongping Zhu, Haiyang Zong, Quwen Li, Sanyan Li
IgG4-related interstitial lung disease (IgG4-RILD), which is characterized by increased IgG4 levels, IgG4 + plasma cell infiltration and irregular whorled fibrosis, is a recently described lung disorder that belongs to the group of systemic fibroinflammatory IgG4-related diseases (IgG4-RD). The aim of the present study was to improve the current knowledge regarding the specific clinical and histopathological characteristics of IgG4-RILD and to investigate its underlying immune mechanism in vivo . A total of 7 patients newly diagnosed with IgG4-RILD were enrolled in the present study (4 men and 3 women; mean age, 57 years; range, 29-71 years)...
February 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29398045/-management-of-orbital-inflammation-in-internal-medicine-proposal-for-a-diagnostic-work-up
#3
S Abad, F Héran, C Terrada, P Bielefeld, D Sène, S Trad, D Saadoun, P Sève
Inflammatory orbitopathies relate to an inflammatory state originating within the orbit and its adnexes, except the inner ocular globe. Orbital inflammation (OI) may be either localized manifestation of a proven or like autoimmune disease, or local response from immune system against infectious, structural or tumoral antigens. We review the clinical manifestations of OI, which provide helpful clues to the diagnosis and describe the inflammatory, infectious and neoplastic conditions classically associated with OI...
February 1, 2018: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29391913/the-utility-of-fdg-pet-ct-in-igg4-related-disease-with-a-focus-on-coronary-artery-involvement
#4
Hian L Huang, Warren Fong, Wee M Peh, Kasat A Niraj, Winnie W Lam
Purpose: Our case series aims to study the growing use of FDG PET/CT in diagnostic evaluation and follow up of IgG4-RD with emphasis on patients presenting with coronary artery involvement. Methods: We conducted a search on the nuclear medicine and rheumatology service databases and identified patients with histologically proven IgG4-RD with FDG PET/CT performed at the Singapore General Hospital. The radiological, clinical, and laboratory findings of these patients were analyzed retrospectively...
February 2018: Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29390345/a-case-of-immunoglobulin-g4-related-lung-disease-with-bilateral-diffuse-infiltration-a-case-report
#5
Ailing Liu, Qianwen Zhang, Bo Liu, Ning Xu, Aijun Li
RATIONALE: Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a rare and chronic progressive autoimmune disease. It is a novel clinical entity characterized by elevated serum IgG4 concentration and tissue infiltration by IgG4 plasma cells. IgG4-related lung disease (IgG4-RLD) has been described as interstitial pneumonia and inflammatory pseudotumor, with various abnormal radiographic patterns. We report a case of IgG4-related lung disease with bilateral diffuse infiltration. PATIENT CONCERNS: A 65-year-old woman was admitted to our hospital because of cough, sputum, and fever...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29388171/combined-membranous-nephropathy-and-tubulointerstitial-nephritis-as-a-rare-renal-manifestation-of-igg4-related-disease-a-case-based-literature-review
#6
Wei Zhang, Jeffrey H Glaze, David Wynne
IgG4-related disease (IgG4-RD) is a newly recognized immune-mediated multisystemic disease characterized by a fibro-inflammatory condition with tissue infiltration of IgG4-positive plasma cells and often associated with elevated serum IgG4 levels. Typical renal involvement of IgG4-RD presents as tubulointerstitial nephritis (TIN), membranous or membranoproliferative nephropathy. We are presenting a case with combined IgG4 membranous nephropathy and TIN, as well as a literature review on pathophysiology, diagnosis and treatment of IgG4-RD...
February 1, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29385874/stage-classification-of-igg4-related-dacryoadenitis-and-sialadenitis-by-the-serum-cytokine-environment
#7
Motohisa Yamamoto, Kenichi Takano, Ryuta Kamekura, Chisako Suzuki, Shingo Ichimiya, Tetsuya Himi, Hiroshi Nakase, Hiroki Takahashi
OBJECTIVES: Patients with immunoglobulin-G4 related disease (IgG4-RD) diagnosed according to the comprehensive diagnostic criteria (CDC) show varied therapeutic responses and prognoses. We assumed that there are clinical stages in IgG4-RD and have verified it using serum cytokine levels in the groups classified by lesion distribution. METHODS: Definite IgG4-related dacryoadenitis and sialadenitis (IgG4-DS) cases were divided according to the CDC for IgG4-RD into 11 cases with focal type and 30 cases with systemic type...
February 1, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29381959/igg4-related-cerebral-pseudotumor-with-perineural-spreading-along-branches-of-the-trigeminal-nerves-causing-compressive-optic-neuropathy-a-case-report
#8
Po-Chang Wu, Peng-Tai Tien, Ying-Hsuan Li, Rui-Yun Chen, Der-Yang Cho
RATIONALE: Immunoglobulin G4-related disease (IgG4-RD) is characterized by tumor-like lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. IgG4-RD has been described in a variety of organ systems; however, it rarely involves the central nervous system. PATIENT CONCERNS: A 17-year-old woman visited our clinic with a complaint of blurred vision for the past 5 months. She also reported a painless right submandibular mass that had been present for 1 year...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29362594/overview-of-igg4-related-disease
#9
R Opriţă, B Opriţă, D Berceanu, I B Diaconescu
Rationale (hypothesis): IgG4-related disease (IgG4-RD) is a pathological entity recently recognized by the medical world that can affect any organ or system. However, there is insufficient data about this disease in medical literature. Aim (objective): A more extensive clarification of the IgG4 molecule, the diversified aspects of IgG4-related disease, and the response of this disease to treatment, will provide a crucial understanding of the immune system and other diseases now known to be associated with IgG4...
October 2017: Journal of Medicine and Life
https://www.readbyqxmd.com/read/29362197/immunoglobulin-g4-related-disease-mimicking-an-anterior-mediastinal-tumor
#10
Seung Ri Kang, Hyeong Ryul Kim, Soo Jeong Nam, Seokchan Hong
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated collection of disorders that were once thought to be unrelated but are increasingly being recognized as a single entity. The tumor-like swelling of the involved organ, a storiform pattern of fibrosis, and a serologic elevation of IgG4 can lead to the diagnosis of IgG4-RD. The usual organs affected are the pancreas, biliary tract, and salivary and lacrimal glands. There have been few reports of intrathoracic cases, which usually have pulmonary lesions...
February 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29359118/immunoglobulin-g4-related-kidney-diseases-an-updated-review
#11
REVIEW
Maurizio Salvadori, Aris Tsalouchos
This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases (IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis...
January 6, 2018: World Journal of Nephrology
https://www.readbyqxmd.com/read/29352044/resolution-of-spurious-immunonephelometric-igg-subclass-measurement-discrepancies-by-lc-ms-ms
#12
Grace van der Gugten, Mari L DeMarco, Luke Y C Chen, Alex Chin, Mollie Caruthers, Daniel T Holmes, Andre Mattman
BACKGROUND: The Binding Site immunonephelometric (IN) IgG subclass reagents (IgG1, IgG2, IgG3, IgG4, BSIN) are used for assessment of both immunodeficiency and IgG4-related disease (IgG4-RD). In our laboratory, suspected analytic errors were noted in patients with increases in IgG4: The sum of the individual IgG subclasses was substantially greater than the measured total IgG concentrations (unlike samples with normal IgG4), and the IgG4 concentration was always less than the IgG2 concentration...
January 19, 2018: Clinical Chemistry
https://www.readbyqxmd.com/read/29349436/superficial-temporal-artery-aneurysm-associated-with-immunoglobulin-g4-related-disease
#13
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/29329570/exophthalmos-in-a-young-woman-with-no-graves-disease-a-case-report-of-igg4-related-orbitopathy
#14
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/29323079/immunoglobulin-g4-related-tubulointerstitial-nephritis-a-not-to-be-missed-diagnosis
#15
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/29310344/polymyositis-with-elevated-serum-igg4-levels-and-abundant-igg4-plasma-cell-infiltration-a-case-report-and-literature-review
#16
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-igg-4-oligoarthritis-mimicking-psoriatic-arthritis
#17
Ziad Farah, Neil Mo
IgG 4 -related disease (IgG 4 -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, IgG 4 -RD presenting primarily as an inflammatory arthritis is much less recognised. We present a rare case of IgG 4 -RD mimicking psoriatic arthritis. In spite of normal circulating IgG 4 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/29289265/utility-of-serum-igg4-levels-in-a-multiethnic-population
#18
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/29285637/lymphomas-in-igg4-related-disease-clinicopathologic-features-in-a-western-population
#19
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/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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