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IgG4- related diseases ( IgG4-RD ) | Page 3

Maia Winkel, Cort D Lawton, Olabisi R Sanusi, Craig M Horbinski, Nader S Dahdaleh, Zachary A Smith
Background: Immunoglobulin G4-related disease (IgG4-RD) is a group of distinct autoimmune disorders affecting nearly every organ system in the body. Although central nervous system involvement is quite rare, it may present as hypertrophic pachymeningitis more frequently affecting the brain than the spine. In this study, we provide a case of spinal IgG4-RD pseudotumor resulting in cord compression, and a comprehensive review of the literature. Case Description: A patient presented with an extradural mass causing spinal cord compression at the L2-L3 level...
2018: Surgical Neurology International
Shoko Matsui
IgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respiratory disease (IgG4-RRD). IgG4-RRD has few subjective symptoms, and is usually detected during workup of patients with extra-thoracic lesions of IgG4-RD. IgG4-RRD is characterized by various conditions, including masses, nodules, thickening, and infiltration at numerous sites in the thorax through lymphatic routes. Although elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells are characteristic findings of IgG4-RD, other intrathoracic diseases, such as multicentric Castleman disease and malignancy, may present with similar findings...
November 24, 2018: Modern Rheumatology
Ryohei Yoshikawa, Hitoshi Igai, Fumi Ohsawa, Tomohiro Yazawa, Mitsuhiro Kamiyoshihara
We treated a patient with immunoglobulin G4-related disease (IgG4-RD) exhibiting an isolated anterior mediastinal mass with high 2-[18 F]-fluoro-2-deoxy-D-glucose (FDG) accumulation, which mimicked thymoma. The 71-year-old male who had a 47 mm anterior mediastinal mass shadow, was referred to our department. As positron emission tomography(PET) revealed the mass to have a high level of FDG accumulation, we diagnosed it as suspected thymoma; thoracoscopic resection of the tumor was then performed. Immunohistological findings, however, yielded a diagnosis of IgG4-RD...
November 21, 2018: Annals of Thoracic Surgery
Atsushi Kitazawa, Ryo Koda, Atsunori Yoshino, Yoshihiko Ueda, Tetsuro Takeda
BACKGROUND: IgG4-related disease (IgG4-RD) is a newly recognized fibroinflammatory condition. The kidney is one of the organs commonly affected by IgG4-RD. Tubulointerstitial nephritis (TIN) is the main feature, and membranous nephropathy (MN) has also been described frequently. In MN, polyclonal immunoglobulins and complements are deposited in granular form along the glomerular basement membranes (GBMs). Unusual cases of monoclonal immunoglobulin deposition disease (MIDD) associated with membranous features have been reported...
November 20, 2018: BMC Nephrology
Martin S Taylor, Abhijit Chougule, Allsion R MacLeay, Pawel Kurzawa, Ivan Chebib, Long Le, Vikram Deshpande
Inflammatory myofibroblastic tumor (IMT), a locally aggressive neoplasm capable of metastasis, may show an immunoglobulin (Ig)G4-rich lymphoplasmacytic infiltrate. Prior reports suggest that storiform-fibrosis and obliterative phlebitis aid in the distinction of IMT from IgG4-related diseases. Herein, we highlight the morphologic overlap between the 2 diseases, and emphasize the importance of a multiplex fusion assay in the distinction of IgG4-related disease (IgG4-RD) from IMT. We identified 7 IMTs with morphologic and immunohistochemical features of IgG4-RD; 3 patients were originally diagnosed with IgG4-RD...
November 16, 2018: American Journal of Surgical Pathology
Jing Xue, Xue-Mei Wang, Yan Li, Li Zhu, Xiao-Ming Liu, Juan Chen, Shu-Hong Chi
IgG4-related disease (IgG4-RD) is an increasingly recognized pathological entity that tends to involve multiple organs with an elevated level of serum IgG4, which is easily misdiagnosed owing to sharing common clinical features with a variety of other diseases. Here, we report an interesting IgG4-RD case of a woman with progressive multi-organ involvement for over 19 years, started with swollen eyelids, dry eye and mouth, and polydipsia and hydruria. Imaging diagnosis revealed diffuse enlargement of the parotid glands, enlargement of the head of the pancreas, pulmonary infection and interstitial lung...
November 6, 2018: World Journal of Clinical Cases
Fahd Touzani, Agnieszka Pozdzik
Immunoglobulin G4-related disease (IgG4-RD) is a newly acknowledged entity, characterized by an immune-mediated fibro-inflammatory process affecting virtually all organs, with infiltration of IgG4+ bearing plasma cells. Until today the pathogenesis of IgG4-RD remains unknown. Treatment with anti-CD20 monoclonal antibodies efficiently induced remission and attenuated the secretory phenotype of myofibroblasts responsible of uncontrolled collagen deposition. This supports the pathogenic role of the adaptive immunity, particularly B cell compartment and B cell/T cell interaction...
November 10, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Kshitij Arora, Miguel Rivera, David T Ting, Vikram Deshpande
INTRODUCTION: The pathologic diagnosis of IgG4-related disease (IgG4-RD) relies on histology, IgG4 positive cells and increased IgG4:IgG. Small biopsies from patients with a presumptive diagnosis of IgG4-RD often fail to meet consensus histologic guidelines. We evaluate consecutive small biopsies from patients with a presumptive diagnosis of IgG4-RD and assess the significance of the pathologic findings. METHODS: We evaluated 55 small biopsies from patients with a presumptive diagnosis of IgG4-RD...
November 8, 2018: Histopathology
Tomohiro Watanabe, Kosuke Minaga, Ken Kamata, Masatoshi Kudo, Warren Strober
Autoimmune pancreatitis (AIP) is a pancreatic manifestation of a recently defined disease form known as IgG4-related disease (AIP/IgG4-RD). AIP/IgG4-RD is characterized by elevated systemic IgG4 antibody concentrations and lesional tissues infiltrated by IgG4-expressing plasmacytes. In addition, recent studies have revealed that, in common with other autoimmune diseases, such as systemic lupus erythematosus (SLE) and psoriasis, AIP/IgG4-RD is associated with increased type I IFN (IFN-I) production by plasmacytoid dendritic cells (pDCs)...
November 2018: Trends in Immunology
Kunio Mochizuki, Yuki Hanai, Kumiko Nakazawa, Shiori Murata, Kazuki Kasai, Naoko Mochizuki, Ippei Tahara, Tetsuo Kondo
IgG4-related disease (IgG4-RD) can affect various organs, and the pancreas and salivary gland are representative examples. We report a rare case of IgG4-RD of the paratestis. A 74-year-old man presented with left scrotal swelling. Scrotopuncture drainage and cytology confirmed a clear, yellow retention liquid (130 mL) with many small, similar lymphocytes and a few plasmacytes. Many lymphoid cells were immunopositive for CD3 on a cell block section, indicating that a predominant type of lymphoid cells was T cell...
October 30, 2018: Diagnostic Cytopathology
Motohisa Yamamoto, Ken-Ichi Takano, Ryuta Kamekura, Satsuki Aochi, Chisako Suzuki, Shingo Ichimiya, Hiroshi Nakase, Tetsuo Himi, Hiroki Takahashi
OBJECTIVES: Immunoglobulin (Ig) G4-related disease (IgG4-RD) is often complicated by allergic disorders. This study was conducted to investigate the mechanism of type 2 helper T-inflammation (Th2-inflammation) in IgG4-related dacryoadenitis and sialadenitis (IgG4-DS). METHODS: We separated and analyzed the proportion of growth stimulation expressed gene 2 (ST2)+ memory Th2 cells among the peripheral blood mononuclear cells by flow cytometry in cases with IgG4-DS and healthy individuals...
October 25, 2018: Modern Rheumatology
Emma L Culver, Fleur S van de Bovenkamp, Ninotska I L Derksen, Jana Koers, Tamsin Cargill, Eleanor Barnes, Louise A de Neef, Carolien A M Koeleman, Rob C Aalberse, Manfred Wuhrer, Theo Rispens
BACKGROUND: IgG4-related disease (IgG4-RD) is characterized by an abundance of IgG4 antibodies in the serum and tissue. Glycosylation status of antibodies can impact on immune effector functions and disease pathophysiology. We sought to establish glycosylation patterns in a prospective cohort of patients with IgG4-RD, and the relationship with disease activity and response to treatment. METHODS: We assessed IgG Fc-tail and Fab-arm glycosylation status in patients with IgG4-RD (n=22), disease controls with primary sclerosing cholangitis (PSC) (n=22), and healthy controls (HCs n=22)...
October 21, 2018: Journal of Gastroenterology and Hepatology
Chuanyin Sun, Guanhua Xu, Jin Lin
BACKGROUND: It can be difficult to distinguish between IgG4-related lymphadenopathy and multicentric Castleman's disease (MCD) because these conditions cannot be differentially diagnosed using immunohistochemical staining alone. In this study, we analyzed the clinical features of IgG4-related lymphadenopathy and MCD patients. METHODS: We retrospectively analyzed 27 patients with MCD, including 20 with plasma cell-type (PC-type) and 7 with hyaline vascular (HV) features (mixed-type)...
October 1, 2018: Clinical Laboratory
Rachel Wallwork, Zachary Wallace, Cory Perugino, Amita Sharma, John H Stone
Retroperitoneal fibrosis (RPF) refers to a fibro-inflammatory lesion in the retroperitoneum, often anterolateral to the aorta. Most cases are due to IgG4-related disease (IgG4-RD) or are idiopathic. RPF can lead to severe morbidity. Treatment strategies remain poorly-defined. We evaluated the efficacy and safety of rituximab (RTX) for idiopathic or IgG4-related RPF.We retrospectively reviewed the records of patients who had RPF treated with RTX. Treatment response was determined by assessing changes in both clinical features, including symptoms and laboratory measurements, as well as in the radiographic dimensions of the lesion...
October 2018: Medicine (Baltimore)
Satoshi Kubo, Shingo Nakayamada, Yoshiya Tanaka
As stated in the comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), IgG4-RD is characterized by elevated serum IgG4 level and pathological findings, characterized by infiltration of IgG4-positive plasma cells. In addition to fibrotic changes, dysregulated activation of lymphocytes is considered as one of major pathogenic events in IgG4-RD. Among lymphocytes, the importance of plasmablast, T follicular helper (Tfh) cells, T type 2 helper (Th2) cells, T regulatory (Treg) cells, and CD4 positive T cells with cytotoxic activity has been reported...
October 18, 2018: Modern Rheumatology
Tomoe Yoshikawa, Tomohiro Watanabe, Kosuke Minaga, Ken Kamata, Masatoshi Kudo
IgG4-related disease (IgG4-RD) is a newly defined multi-organ disease proposed by Japanese physicians. IgG4-RD is characterized by elevated serum levels of IgG4 and massive infiltration of IgG4-expressing plasma cells in the affected organs. Recent studies have shown that abnormal adaptive immune responses mediated by T helper type 2 cells, regulatory T cells, follicular helper T cells, cytotoxic CD4+ T cells, and plasmablasts are involved in IgG4-RD immunopathogenesis. In addition to adaptive immune responses, innate immune responses play pathogenic roles in IgG4-RD...
October 18, 2018: Modern Rheumatology
Takahiro Kawakami, Ichiro Mizushima, Kazunori Yamada, Hiroshi Fujii, Kiyoaki Ito, Tetsuhiko Yasuno, Shozo Izui, Masakazu Yamagishi, Bertrand Huard, Mitsuhiro Kawano
Background: This study aimed to investigate the contribution of a proliferation-inducing ligand (APRIL), a member of the tumor necrosis factor (TNF) superfamily implicated in plasma cell survival, to the development of plasma cell-rich lesions in immunoglobulin G4-related disease (IgG4-RD). Methods: We performed immunohistochemical staining for APRIL with Stalk-1 and Aprily-8 antibodies specifically recognizing APRIL-producing cells and secreted APRIL, respectively, in renal and submandibular lesions of IgG4-RD in comparison with those of Sjögren's syndrome and sialolithiasis...
October 15, 2018: Nephrology, Dialysis, Transplantation
Matthew A Weiss, Marie-Christine Aubry, Benjamin R Pflederer
BACKGROUND IgG4-related disease (IgG4-RD) is a systemic inflammatory condition with a myriad of presentations related to the pattern of organ involvement. Diagnostic workup for IgG4-RD requires a high index of suspicion, and further workup often includes the results of serological testing for elevated levels of IgG4. Correlation of presentation, past medical history, and histopathologic analysis are required to make a diagnosis. CASE REPORT In this case, incidental discovery of non-infectious aortitis and pulmonary mass lesions were the specific signs that led to the consideration of IgG4-RD...
October 16, 2018: American Journal of Case Reports
Shahine Goulam-Houssein, Jeffrey L Grenville, Katerina Mastrocostas, David G Munoz, Amy Lin, Aditya Bharatha, Paraskevi A Vlachou
IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG4 pachymeningitis mimicking a sphenoid wing meningioma, which is to our knowledge the largest mass-forming pachymeningitis published in the literature...
February 2019: Neuroradiology Journal
L Yang, C Q Yang
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease that share common pathologic, serologic and clinical features. IgG4- RD may include inflammatory pseudotumor, IgG4-related autoimmune hepatitis, and type 1 autoimmune pancreatitis mainly involving liver and clinically classified into three types. IgG4-related sclerosing cholangitis is a rare disease. It is frequently present in association with type 1 autoimmune pancreatitis, so it needs to be distinguishing from primary sclerosing cholangitis...
June 20, 2018: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
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