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IgA vasculitis

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https://www.readbyqxmd.com/read/29438165/histologic-features-of-gastrointestinal-tract-biopsies-in-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura
#1
Christine Y Louie, Adam J Gomez, Richard K Sibley, Dorsey Bass, Teri A Longacre
Immunoglobulin A (IgA) vasculitis or Henoch-Schönlein purpura (HSP) typically occurs in the pediatric population, although rare cases also occur in adults. Gastrointestinal (GI) involvement is common. The "classic" histologic finding in IgA vasculitis (HSP) is leukocytoclastic vasculitis (LCV); other histologic features in biopsies of IgA vasculitis (HSP) have only been rarely described. The pathology archival files at our institution were searched for GI biopsies from patients with IgA vasculitis (HSP)...
February 12, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29409373/epidemiology-of-primary-systemic-vasculitis-in-children-a-population-based-study-from-southern-sweden
#2
M Mossberg, M Segelmark, R Kahn, M Englund, A J Mohammad
OBJECTIVES: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden. METHODS: Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register...
February 7, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29374629/cross-phenotype-analysis-of-immunochip-data-identifies-kdm4c-as-a-relevant-locus-for-the-development-of-systemic-vasculitis
#3
Lourdes Ortiz-Fernández, Francisco David Carmona, Raquel López-Mejías, Maria Francisca González-Escribano, Paul A Lyons, Ann W Morgan, Amr H Sawalha, Kenneth G C Smith, Miguel A González-Gay, Javier Martín
OBJETIVE: Systemic vasculitides represent a heterogeneous group of rare complex diseases of the blood vessels with a poorly understood aetiology. To investigate the shared genetic component underlying their predisposition, we performed the first cross-phenotype meta-analysis of genetic data from different clinically distinct patterns of vasculitis. METHODS: Immunochip genotyping data from 2465 patients diagnosed with giant cell arteritis, Takayasu's arteritis , antineutrophil cytoplasmic antibody-associated vasculitis or IgA vasculitis as well as 4632 unaffected controls were analysed to identify common susceptibility loci for vasculitis development...
January 27, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29329643/iga-nephropathy-and-iga-vasculitis-with-nephritis-have-a-shared-feature-involving-galactose-deficient-iga1-oriented-pathogenesis
#4
Hitoshi Suzuki, Junichi Yasutake, Yuko Makita, Yuki Tanbo, Kohei Yamasaki, Tadashi Sofue, Toshiki Kano, Yusuke Suzuki
Galactose-deficient IgA1 has been proposed as an important effector molecule in IgA nephropathy (IgAN). We previously showed that the galactose-deficient IgA1-specific monoclonal antibody KM55 can detect circulating galactose-deficient IgA1 in patients with IgAN, enabling us to study the molecular roles of galactose-deficient IgA1. Herein, we further examined the pathophysiological significance of galactose-deficient IgA1 in glomerular deposits of patients with IgAN by immunohistochemistry using KM55. Immunostaining of galactose-deficient IgA1 with KM55 was performed in paraffin-embedded sections of renal biopsy specimens from 48 patients with IgAN and 49 patients with other renal diseases such as lupus nephritis, HCV-related nephropathy, IgA vasculitis with nephritis (IgA-VN), and membranous nephropathy...
January 9, 2018: Kidney International
https://www.readbyqxmd.com/read/29319773/anca-positive-pauci-immune-crescentic-glomerulonephritis-in-a-patient-with-systemic-lupus-erythematosus
#5
Percy Herrera-Añazco, Percy Velásquez-Castillo, Josmel Pacheco-Mendoza, Germán Valenzuela-Rodriguez, Carmen Asato-Higa
The pauci-immune crescentic glomerulonephritis (PICGN) is generally associated with small-vessel vasculitis with a few reported cases associated with other autoimmune diseases such as Systemic Lupus Erythematosus (SLE). We present the case of a female 34-year-old patient with acute kidney injury symptoms with indication for renal replacement therapy in the context of clinical SLE diagnosis. A kidney biopsy was conducted and it was found that most glomeruli showed some segmental sclerosis with synechia to the Bowman's capsule...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29304917/ultraviolet-purpura-in-iga-vasculitis
#6
Daisuke Tsukui, Hajime Kono
No abstract text is available yet for this article.
January 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29287933/-humoral-immune-diseases-cutaneous-vasculitis-and-auto-immune-bullous-dermatoses
#7
Janine Wechsler
Humoral immunity is the cause of multiple diseases related to antibodies (IgA, IgG, IgM) produced by the patient. Two groups of diseases are identified. The first group is related to circulating antigen-antibody complexes. The antigens are various. They are often unknown. These immune complexes cause a vascular inflammation due to the complement fixation. Consequently, this group is dominated by inflammatory vasculitis. In the second group, the pathology is due to the fixation in situ of antibodies to a target antigen of the skin that is no more recognized by the patient...
December 26, 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/29279496/a-case-of-paraneoplastic-iga-vasculitis-in-an-adult-with-lung-adenocarcinoma
#8
Shuji Ota, Terunobu Haruyama, Masashi Ishihara, Maika Natsume, Yoko Fukasawa, Takahiko Sakamoto, Shigeru Tanzawa, Ryo Usui, Takeshi Honda, Yasuko Ichikawa, Kiyotaka Watanabe, Yuko Sasajima, Nobuhiko Seki
A 50-year-old man with lung adenocarcinoma (c-T1aN2M1b) experienced reddish purpura mainly on the lower legs after receiving 12 cycles of second-line chemotherapy with docetaxel. There was tumor enlargement on computed tomography performed to assess the therapeutic response, so paraneoplastic IgA vasculitis was considered. IgA vasculitis was diagnosed based on a biopsy of the skin lesion and histology of an upper gastrointestinal hemorrhagic mucosal erosion. As IgA vasculitis can lead to serious gastrointestinal or systemic complications, IgA vasculitis should be considered as a differential diagnosis for rashes in patients with malignancy...
December 27, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29241772/location-of-skin-lesions-in-henoch-sch%C3%A3-nlein-purpura-and-its-association-with-significant-renal-involvement
#9
COMPARATIVE STUDY
Jessica St John, Priyanka Vedak, Anna Cristina Garza-Mayers, Mai P Hoang, Sagar U Nigwekar, Daniela Kroshinsky
BACKGROUND: Henoch-Schönlein purpura (HSP) is a small vessel IgA-predominant vasculitis. OBJECTIVE: To describe adult patients with HSP and determine if the distribution of skin lesions (ie, purpura above the waist or purpura below the waist only), is a predictor of significant renal involvement at the time of the skin biopsy and the months following. METHODS: A retrospective study on renal function from 72 adult patients with skin-biopsy proven HSP...
January 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29236760/distribution-of-circulating-t-follicular-helper-cell-subsets-is-altered-in-immunoglobulin-a-vasculitis-in-children
#10
Deying Liu, Jinxiang Liu, Jinghua Wang, Lishuang Guo, Congcong Liu, Yanfang Jiang, Haifeng Wang, Sirui Yang
BACKGROUND: Immunoglobulin A vasculitis (IgAV) is the most common vasculitis in children. Previously, we demonstrated that patients with IgAV show abnormal proliferation of cluster of differentiation (CD)4+C-X-C chemokine receptor type (CXCR)5+ follicular helper T (Tfh) cells. Here, we explored the status of Tfh cell subsets and plasma cytokine levels in patients with IgAV. METHODS: CD4+CXCR5+CD45RA-, CD45RA-CXCR3+CCR6-, CD45RA-CXCR3-CCR6-, CD45RA-CXCR3-CCR6+, and CD45RA-CXCR3+CCR6+ Tfh cell fractions and plasma concentrations of interferon-γ, interleukin (IL)-4, and IL-17A were evaluated by flow cytometry and a flow cytometric bead array, respectively, in 30 patients with IgAV and 15 healthy controls (HCs)...
2017: PloS One
https://www.readbyqxmd.com/read/29225270/iga-vasculitis-with-simultaneous-cardiopulmonary-involvement-a-case-report
#11
Kazunori Bando, Hirofumi Maeba, Ichiro Shiojima
A 60-year-old man with a history of hypertension, type 2 diabetes, and reflux esophagitis was admitted to our hospital with hemoptysis, dyspnea, and leg edema. We diagnosed him with adult IgA vasculitis based on the presence of purpura, elevated serum IgA fibronectin complexes, pathophysiological findings, a skin biopsy showing leukocytoclastic vasculitis, and immunofluorescence studies demonstrating granular IgA and C3 deposits in the blood vessel wall. He showed concurrent cardiopulmonary involvement without involvement of the gastrointestinal system and kidneys, which are commonly affected in IgA vasculitis patients...
December 8, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29219653/characteristics-and-outcome-of-intractable-vasculitis-syndrome-in-children-nation-wide-survey-in-japan
#12
Naoko Nakano, Masaaki Mori, Hiroaki Umebayashi, Naomi Iwata, Norimoto Kobayashi, Kenji Masunaga, Tomoyuki Imagawa, Takuji Murata, Noriko Kinjo, Kazushige Nagai, Mari Miyoshi, Syuji Takei, Shumpei Yokota, Eiichi Ishii
OBJECTIVE: Primary systemic vasculitis (PSV) is a rare disorder in children and difficult to distinguish from other diseases. However, appropriate diagnosis and prompt treatment will affect on the morbidity and mortality of intractable PSV. In this study, we conducted a nationwide survey in Japan, to clarify epidemiology and clinical outcome of PSV. METHODS: We had sent survey questionnaires to most of the Japanese institutions that employed pediatricians, requesting the number of patients with refractory PSV who were diagnosed and treated between 2007 and 2011...
December 8, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29204761/clinical-value-of-systemic-symptoms-in-iga-nephropathy-with-anca-positivity
#13
Lijiao Xie, Jianghua He, Xing Liu, Sha Tang, Weili Wang, Furong Li, Ying Zhang, Jun Zhang, Yunjian Huang, Jinghong Zhao, Yafei Li, Jingbo Zhang
Our aim was to evaluate the pathogenic role of anti-neutrophil cytoplasmic antibodies (ANCAs) in patients with IgA nephropathy (IgAN). A total of 2390 patients with biopsy-confirmed IgAN were analyzed retrospectively. Thirty-five IgAN patients with ANCA and 40 IgAN patients without ANCA were enrolled. According to the Birmingham Vasculitis Activity Score (BVAS) items, the ANCA-positive patients were further divided into two subgroups which with or without systemic symptoms. The cumulative renal survival rate was calculated using Kaplan-Meier analysis...
December 4, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29190714/biomarkers-of-iga-vasculitis-nephritis-in-children
#14
Evangeline Pillebout, Agnès Jamin, Hamza Ayari, Pierre Housset, Melissa Pierre, Virginia Sauvaget, Denis Viglietti, Georges Deschenes, Renato C Monteiro, Laureline Berthelot
Henoch-Schönlein purpura is a systemic vasculitis characterized by IgA deposits, which target the skin, joints, and kidneys, among other organs. In children, prognosis is often good but little is known about biomarkers of pediatric nephritis. We hypothesized that biological markers, including cytokines, immunoglobulins, IgA-immune complexes, IgA glycosylation and neutrophil gelatinase-associated lipocalin (NGAL), may discriminate IgA vasculitis (IgAV) pediatric patients with renal involvement from those without renal involvement...
2017: PloS One
https://www.readbyqxmd.com/read/29189089/interferon-inducible-mx1-protein-is-highly-expressed-in-renal-tissues-from-treatment-na%C3%A3-ve-lupus-nephritis-but-not-in-those-under-immunosuppressive-treatment
#15
Yuka Shimizu, Shinsuke Yasuda, Taichi Kimura, Saori Nishio, Michihiro Kono, Kazumasa Ohmura, Sanae Shimamura, Michihito Kono, Yuichiro Fujieda, Masaru Kato, Kenji Oku, Toshiyuki Bohgaki, Yuichiro Fukasawa, Shinya Tanaka, Tatsuya Atsumi
OBJECTIVES: The aim of this study was to clarify the consequences of Mx1, one of the IFN-inducible proteins, in the peripheral blood as well as in renal tissues in patients with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: Mx1 protein concentrations in (PBMCs) from 18 SLE patients mostly in their stable disease status, 11 IgA nephropathy (IgAN) patients, 5 ANCA-associated vasculitis (AAV) patients and 16 healthy controls were measured using enzyme-linked immunosorbent assay (ELISA)...
November 30, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29162292/posterior-reversible-encephalopathy-syndrome-in-iga-vasculitis-neuroimaging-of-a-14-year-old-child
#16
Harun Arslan, Alpaslan Yavuz, Ayşe Arslan, Abdurrahman Aycan
IgA vasculitis (IgAV) is a leukocytoclastic vasculitis and characterized by involvement of small vessels in skin, gastrointestinal system, joints, kidneys, and less frequently other organs. It is the commonest vasculitis in childhood and etiology is not completely known. Neurological manifestations of IgAV are very rare and usually seen in patients with severe hypertension or as an uncommon feature such as peripheral neuropathy. Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiologic entity characterized with temporary vasogenic edema developing typically in posterior circulation of the brain and has been reported as a rare manifestation of IgAV...
November 14, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29136340/dermatological-addendum-to-the-2012-international-chapel-hill-consensus-conference-nomenclature-of-vasculitides
#17
Cord H Sunderkötter, Bernhard Zelger, Ko-Ron Chen, Luis Requena, Warren Piette, J Andrew Carlson, Jan Dutz, Peter Lamprecht, Alfred Mahr, Elisabeth Aberer, Victoria P Werth, David A Wetter, Seiji Kawana, Raashid Luqmani, Camille Frances, Joseph Jorizzo, J Richard Watts, Dieter Metze, Marzia Caproni, Erkan Alpsoy, Jeffrey P Callen, David Fiorentino, Peter A Merkel, Ronald J Falk, J Charles Jennette
OBJECTIVE: The objective of this project was to prepare a dermatologic addendum to the 2012 Chapel Hill consensus conference nomenclature to address vasculitides affecting the skin. The goal was to standardize names and definitions for cutaneous vasculitis. METHODS: A nominal group technique with a facilitator was used to reach a consensus using multiple face-to-face meetings, e-mail discussions, and teleconferences. RESULTS: Standardized names, definitions and descriptions are provided for cutaneous components of systemic vasculitides (e...
November 14, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29126311/value-of-biomarkers-for-predicting-immunoglobulin-a-vasculitis-nephritis-outcome-in-an-adult-prospective-cohort
#18
Laureline Berthelot, Agnès Jamin, Denis Viglietti, Jonathan M Chemouny, Hamza Ayari, Melissa Pierre, Pierre Housset, Virginia Sauvaget, Margarita Hurtado-Nedelec, François Vrtovsnik, Eric Daugas, Renato C Monteiro, Evangeline Pillebout
Background: Henoch-Schönlein purpura, more recently renamed immunoglobulin A vasculitis (IgAV), is a systemic vasculitis characterized by IgA deposits. The current markers used to assess IgAV inaccurately evaluate the risk of nephritis occurrence and its long-term outcomes. The current study assessed biomarkers of nephritis outcomes. Methods: This French multicentre prospective study enrolled 85 adult patients at the time of disease onset. Patients were assessed for clinical and biological parameters and re-examined after 1 year...
November 3, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29117463/vasculitis-and-iga-monoclonal-gammopathy-of-cutaneous-significance
#19
L Rousset, F Cordoliani, M Battistella, M Jachiet, E Pillebout, M Rybojad, M Bagot, E Oksenhendler, J-D Bouaziz
IgA vasculitis (Henoch-Schonlein purpura) is a small vessel systemic vasculitis involving IgA immunoglobulin polyclonal activation(1) . Monoclonal gammopathy of cutaneous significance is a subgroup of monoclonal gammopathy with skin disease, without myeloma or lymphoproliferative disorder(2) . We report 3 patients with vasculitis and IgA monoclonal gammopathy of cutaneous significance. Three men, 38, 37 and 50 years old, had a vascular purpura for more than 10 years with partial remission and frequent necrotic flares (Figure 1)...
November 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29106654/the-population-level-costs-of-immunosuppression-medications-for-the-treatment-of-glomerulonephritis-are-increasing-over-time-due-to-changing-patterns-of-practice
#20
Sean Barbour, Clifford Lo, Gabriela Espino-Hernandez, Sharareh Sajjadi, John Feehally, Scott Klarenbach, Jagbir Gill
Background: Immunosuppression (IS) is the main treatment for most types of glomerulonephritis (GN). Quantifying the cost of IS is necessary to ensure equitable access to therapies and optimal health outcomes, but the real-world cost of IS treatment for GN is largely unknown. We examined temporal changes in the population-level IS medication costs for GN over a 14-year period in a large Canadian province. Methods: We linked a provincial pathology database (containing all GN cases from 2000 to 2012) with renal and medication administrative databases to capture clinical characteristics and IS medications, with follow-up until 2013...
July 2, 2017: Nephrology, Dialysis, Transplantation
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