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IgA vasculitis

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https://www.readbyqxmd.com/read/29225270/iga-vasculitis-with-simultaneous-cardiopulmonary-involvement-a-case-report
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/29097818/dapsone-treatment-is-efficient-against-persistent-cutaneous-and-gastrointestinal-symptoms-in-children-with-henoch-sch%C3%A3-nlein-purpura
#11
Jana Volejnikova, Jaroslav Horacek, Frantisek Kopriva
BACKGROUND: Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and by the deposition of IgA immune complexes. An association between HSP and atypical bacteria is uncommon in children. METHODS AND RESULTS: Here we report three cases of children, aged 5, 4 and 16 years, who were diagnosed with HSP associated with Mycoplasma pneumoniae or Chlamydia pneumoniae infection. In all presented cases, persistent cutaneous manifestations and abdominal pain were resistant to antibiotics and corticosteroids, but resolved during 48 h after the introduction of dapsone...
November 2, 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/29092674/autoantibodies-against-complement-components-in-systemic-lupus-erythematosus-role-in-the-pathogenesis-and-clinical-manifestations
#12
M H Hristova, V S Stoyanova
Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Genetic defects in the complement as well as functional deficiency due to antibodies against its components lead to different pathological conditions, usually clinically presented. Among them hypocomplementemic urticarial vasculitis, different types of glomerulonephritis as dense deposit disease, IgA nephropathy, atypical haemolytic uremic syndrome and lupus nephritis are very common...
December 2017: Lupus
https://www.readbyqxmd.com/read/29055398/dapsone-as-a-potential-treatment-option-for-henoch-sch%C3%A3-nlein-purpura-hsp
#13
Keum Hwa Lee, Jae Hyon Park, Dong Hyun Kim, Jimin Hwang, Goeun Lee, Jae Seok Hyun, Sung Taik Heo, Ji Hoon Choi, Minwoo Kim, Minhye Kim, Seong Il Kim, Michael Eisenhut, Andreas Kronbichler, Jae Il Shin
Henoch-Schönlein Purpura (HSP, IgA vasculitis) is an immunoglobulin A (IgA) mediated disorder characterized by systemic vasculitis with variable presentation, frequently affecting the skin, mucous membrane, joints, kidneys, and rarely lungs and the central nervous system. Interestingly, enhanced production of interleukin-8 (IL-8) levels are found during active disease and increased levels have been reported in supernatants from human umbilical venous endothelial cells after stimulation with sera from patients affected by HSP...
October 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/29051623/vasculitis-syndromes-rituximab-for-adult-onset-iga-vasculitis
#14
Joanna Collison
No abstract text is available yet for this article.
December 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29037908/new-insights-in-the-pathogenesis-of-immunoglobulin-a-vasculitis-henoch-sch%C3%A3-nlein-purpura
#15
REVIEW
Marieke H Heineke, Aranka V Ballering, Agnès Jamin, Sanae Ben Mkaddem, Renato C Monteiro, Marjolein Van Egmond
Immunoglobulin A vasculitis (IgAV), also referred to as Henoch-Schönlein purpura, is the most common form of childhood vasculitis. The pathogenesis of IgAV is still largely unknown. The disease is characterized by IgA1-immune deposits, complement factors and neutrophil infiltration, which is accompanied with vascular inflammation. Incidence of IgAV is twice as high during fall and winter, suggesting an environmental trigger associated to climate. Symptoms can resolve without intervention, but some patients develop glomerulonephritis with features similar to IgA nephropathy that include hematuria, proteinuria and IgA deposition in the glomerulus...
October 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29033424/diffuse-alveolar-hemorrhage-in-iga-vasculitis-with-an-atypical-presentation
#16
Yuhei Ito, Machiko Arita, Shogo Kumagai, Reoto Takei, Maki Noyama, Fumiaki Tokioka, Takumi Nagamoto, Chieko Kawakita, Kenichiro Asano, Chika Okita, Tadashi Ishida
IgA vasculitis (IgAV) commonly occurs in young children, who present with a tetrad of purpura, abdominal pain, arthralgia and nephritis. Diffuse alveolar hemorrhage (DAH) is a rare complication of IgAV. We herein report an adult case of IgAV with a presentation of DAH and nephritis (pulmonary renal syndrome, PRS), but without other typical manifestations, such as purpura, abdominal pain and arthralgia. A 33-year-old man presented with hemoptysis and a low-grade fever and was diagnosed to have IgAV based on the results of a renal biopsy...
October 16, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28984999/direct-immunofluorescence-testing-in-vasculitis-a-single-institution-experience-with-henoch-sch%C3%A3-nlein-purpura
#17
Patrick Feasel, Steven D Billings, Wilma F Bergfeld, Melissa P Piliang, Anthony P Fernandez, Jennifer S Ko
BACKGROUND: Direct immunofluorescence (DIF) panels (IgG, IgA, IgM, C3 and fibrinogen) are ordered for clinically suspected vasculitis, with frequently negative results. METHODS: Cases submitted for DIF and histology (2010-2014) with "vasculitis" in the clinical data were examined, and the electronic medical record reviewed for clinical suspicion of HSP. Peri/intravascular IgA was considered "positive," other reactants "non-specific," and no immunoreactivity "negative...
October 6, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28973844/rituximab-for-the-treatment-of-adult-onset-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura
#18
Federica Maritati, Roberta Fenoglio, Evangeline Pillebout, Giacomo Emmi, Maria L Urban, Rossana Rocco, Maria Nicastro, Monia Incerti, Matteo Goldoni, Giorgio Trivioli, Elena Silvestri, Aladdin J Mohammad, David Jayne, Per Eriksson, Mårten Segelmark, Pavel Novikov, Helen Harris, Dario Roccatello, Augusto Vaglio
OBJECTIVE: Adult-onset IgA vasculitis (IgAV, formerly Henoch-Schönlein purpura) is a rare systemic vasculitis characterised by IgA1-dominant deposits. The treatment of adult-onset IgAV is controversial and is based on the combination of glucocorticoids and immunosuppressive agents, but many patients have refractory or relapsing disease despite treatment. Rituximab is a B cell-depleting antibody of proven efficacy in anti-neutrophil cytoplasmic antibody-associated vasculitis. We tested the efficacy and safety of rituximab in a multicentre cohort of patients with adult-onset IgAV...
October 3, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28957963/an-update-on-the-role-of-epigenetics-in-systemic-vasculitis
#19
Patrick Coit, Haner Direskeneli, Amr H Sawalha
PURPOSE OF REVIEW: The purpose of this review is to discuss recent observations of epigenetic changes related to the complex pathogenesis of systemic vasculitides and their contribution to the field. RECENT FINDINGS: There have been new observations of epigenetic changes in vasculitis and their potential role in disease pathogenesis in antineutrophil cytoplasmic antibody-associated vasculitis, giant-cell arteritis, Kawasaki disease, Behçet's disease, and IgA vasculitis...
September 27, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28954579/an-exceptional-cause-of-hemoptysis-in-the-elderly-patient-iga-vasculitis
#20
Aline Pourcelet, Marine Georgery, Frederic Vandergheynst, Jean-Michel Hougardy, Sandra De Breucker
We describe here the case of a 73-year-old woman who presented a recurrent macular rash, acute respiratory distress, and hemoptysis. Chest CT scan showed diffuse ground-glass opacities that were suggestive of alveolar hemorrhage. With the development of severe acute kidney injury and nephrotic-range proteinuria (creatininemia 2.6 from 1.9 mg/dL with overt proteinuria 34 from 2.1 g/g creat), a kidney-lung syndrome was evoked. Skin biopsy revealed leukocytoclastic vasculitis with IgA deposits. Blood tests showed an increased IgA level...
September 28, 2017: Acta Clinica Belgica
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