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Crescentic glomerulonephritis & small vessel vasculitis

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54 papers 0 to 25 followers
By P O Pediatrics, Nephrology
M Yates, R A Watts, I M Bajema, M C Cid, B Crestani, T Hauser, B Hellmich, J U Holle, M Laudien, M A Little, R A Luqmani, A Mahr, P A Merkel, J Mills, J Mooney, M Segelmark, V Tesar, K Westman, A Vaglio, N Yalçındağ, D R Jayne, C Mukhtyar
In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate...
September 2016: Annals of the Rheumatic Diseases
Yoshihiro Arimura, Eri Muso, Shoichi Fujimoto, Midori Hasegawa, Shinya Kaname, Joichi Usui, Toshiko Ihara, Masaki Kobayashi, Mitsuyo Itabashi, Kiyoki Kitagawa, Junichi Hirahashi, Kenjiro Kimura, Seiichi Matsuo
No abstract text is available yet for this article.
June 2016: Clinical and Experimental Nephrology
Kenan Barut, Sezgin Şahin, Amra Adroviç, Özgür Kasapçopur
All inflammatory changes in the vessel wall are defined as vasculitis. Pediatric vasculitis may present with different clinical findings. Although Henoch-Schönlein purpura which is the most common pediatric vasculitis generally recovers spontaneously, it should be monitorized closely because of the risk of renal failure. Although Kawasaki disease is easy to diagnose with its classical findings, the diagnosis may be delayed in case of incomplete Kawasaki disease. Kawasaki disease should be considered especially in infants in case of prolonged fever even if the criteria are not fully met and intravenous immunoglobulin treatment should be administered without delay in order to prevent development of coronary artery aneurism...
December 2015: Türk Pediatri Arşivi
Mouhamadou Moustapha Cisse, Daher Abdoul Karim Omar, Jean De Dieu Nzambaza, Sidy Ba, Awa Cheikh Ndao, Abibatou Sall, Cherif Mouhamed Dial, Maria Faye, El Hadji Fary Ka, Moustapha Faye, Ahmed Tall Lemrabott, Abdou Niang, Boucar Diouf
INTRODUCTION: We reported a case of hemophagocytic syndrome complicating microscopic polyangitis presented by crescentic glomerulonephritis. CASE PRESENTATION: A 22-year-old female patient originated from Dakar, Senegal presented with nephrotic syndrome and rapidly progressive glomerulonephritis. On physical examination, we noticed hyperchromic diffuse punctilious purpura skin lesions predominant on the trunk, the neck and the upper thigh. Immunology investigations revealed strongly positive anti SSA/Ro and anti-SSB...
November 2015: Nephro-urology Monthly
Antoine Kimmoun, Elisabeth Baux, Vincent Das, Nicolas Terzi, Patrice Talec, Pierre Asfar, Stephan Ehrmann, Guillaume Geri, Steven Grange, Nadia Anguel, Alexandre Demoule, Anne Sophie Moreau, Elie Azoulay, Jean-Pierre Quenot, Julie Boisramé-Helms, Guillaume Louis, Romain Sonneville, Nicolas Girerd, Nicolas Ducrocq, Nelly Agrinier, Denis Wahl, Xavier Puéchal, Bruno Levy
BACKGROUND: The outcomes of patients admitted to the intensive care unit (ICU) for acute manifestation of small-vessel vasculitis are poorly reported. The aim of the present study was to determine the mortality rate and prognostic factors of patients admitted to the ICU for acute small-vessel vasculitis. METHODS: This retrospective, multicenter study was conducted from January 2001 to December 2014 in 20 ICUs in France. Patients were identified from computerized registers of each hospital using the International Classification of Diseases, Ninth Revision (ICD-9)...
January 26, 2016: Critical Care: the Official Journal of the Critical Care Forum
Rennie L Rhee, Susan L Hogan, Caroline J Poulton, Julie Anne G McGregor, J Richard Landis, Ronald J Falk, Peter A Merkel
OBJECTIVE: It is still not clear how advances in the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have impacted long-term outcomes. We undertook this study to examine changes over 25 years in long-term clinical outcomes, including the impact of renal function at diagnosis (a potential marker of time to disease detection) and the duration of cyclophosphamide use in AAV patients with renal involvement. METHODS: We included ANCA-positive patients with biopsy-proven AAV diagnosed between 1985 and 2009 who were followed up in the Glomerular Disease Collaborative Network inception cohort...
July 2016: Arthritis & Rheumatology
Amélie Simon, Jean-François Subra, Philippe Guilpain, Pascale Jeannin, Pascale Pignon, Simon Blanchard, Erwan Garo, Sébastien Jaillon, Alain Chevailler, Gilles Renier, Xavier Puéchal, Barbara Bottazzi, Alberto Mantovani, Yves Delneste, Jean-François Augusto
OBJECTIVES: Pentraxin 3 (PTX3), in common with myeloperoxidase and proteinase 3, is stored in human neutrophil granules and is expressed on apoptotic neutrophil surface. We therefore investigated the presence of anti-PTX3 autoantibodies (aAbs) in the sera of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients. METHODS: Presence of anti-PTX3 autoantibodies was analysed by a specific enzyme-linked immunosorbent assay in sera from 150 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA), and in sera of 227 healthy subjects (HS), 40 systemic sclerosis (SSc) patients, and 25 giant cell arteritis patients (GCA)...
2016: PloS One
Wisit Cheungpasitporn, Claudia C Zacharek, Fernando C Fervenza, Lynn D Cornell, Sanjeev Sethi, Loren P Herrera Hernandez, Samih H Nasr, Mariam P Alexander
Anti-glomerular basement membrane (anti-GBM) disease is a major cause of rapidly progressive glomerulonephritis (RPGN). On the other hand, fibrillary glomerulonephritis (GN) typically presents as proteinuria, hematuria and renal insufficiency, but rarely as RPGN. Without electron microscopy, the diagnosis of fibrillary GN can be missed. We report a 68-year-old white woman who presented with RPGN with kidney biopsy demonstrating diffuse crescentic GN on light microscopy. By immunofluorescence, there was bright linear staining of the GBMs and smudgy mesangial staining for immunoglobulin G, C3, and kappa and lambda light chain...
February 2016: Clinical Kidney Journal
Kai-Hsiang Shu, Wen-Chih Chiang, Yen-Ling Chiu
No abstract text is available yet for this article.
April 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
Bethel Shiferaw, Viktor Miro, Carroll Smith, Jagadish Akella, Walter Chua, Zae Kim
Goodpasture's disease is an uncommon composite of features including renal failure with pulmonary hemorrhage secondary to an autoimmune response that specifically targets these organ systems. We present a case of particular interest in regards to atypical presentation, and the uncommon treatment that the patient underwent. A 65-year-old Afghani female arrived with complaints of nausea, vomiting, loss of appetite, malaise, decreased urine output, exertional dyspnea, and cough. The patient presented initially with renal failure and unexpectedly developed respiratory failure after hemodialysis...
January 2016: Journal of Clinical Medicine Research
Giuseppe A Ramirez, Miriam Blasi, Clara Sciorati, Patrizia Rovere-Querini, Angelo A Manfredi
Anti-Neutrophil Cytoplasmic Antibodies (ANCA)-associated vasculitides (AAV) are characterized by small vessel injury and in some cases granulomatous lesions and glomerular inflammation. The pathogenic bases of these clinical phenotypes are incompletely understood, but evidence from patients with AAV and other inflammatory diseases suggest a role for monocyte/macrophages in the perpetuation of tissue injury. Macrophage colony stimulating factor (M-CSF) is a promoter of monocyte recruitment and macrophage proliferation, involved in mesangial cell proliferation and experimental nephritis development...
2015: Results in Immunology
A Greco, C Marinelli, M Fusconi, G F Macri, A Gallo, A De Virgilio, G Zambetti, M de Vincentiis
Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility...
June 2016: International Journal of Immunopathology and Pharmacology
William G Couser, Richard J Johnson
O'Sullivan et al. describe glomerular localization of myeloperoxidase (MPO) in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and correlate the amount of deposition with severity of injury. MPO is the antigen against which anti-MPO ANCAs are directed, the antigen to which pathogenic T cells that can induce antibody-independent AAV are directed, and MPO can induce glomerular injury directly by interacting with H2O2 and a halide to halogenate glomerular structures. All three of these roles are likely involved in human disease...
November 2015: Kidney International
Cees G M Kallenberg
PURPOSE OF REVIEW: Antineutrophil cytoplasmic autoantibodies (ANCAs) are considered important diagnostic tests in the work-up of patients suspected of vasculitis. Here we discuss new developments in the methodology of testing, the pitfalls in using these tests as diagnostic tools, and the value of serial ANCA testing for the follow-up of patients with ANCA-associated vasculitis including treatment decisions. RECENT FINDINGS: Both the indirect immunofluorescence (IIF) test and antigen-specific assays should be performed...
January 2016: Current Opinion in Rheumatology
Lindsay Lally, Robert Spiera
PURPOSE OF REVIEW: The present review discusses the evidence supporting the use of B-cell-targeted therapy in the treatment of various forms of systemic vasculitis with a focus on the use of rituximab (RTX), in the antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV). RECENT FINDINGS: Long-term follow-up of the two studies establishing the efficacy of a RTX-based induction regimen for severe AAV have demonstrated noninferiority of a single course of RTX compared with conventional therapy for remission maintenance...
January 2016: Current Opinion in Rheumatology
Neeraj Dhaun, Andrew Saunders, Christopher O Bellamy, Rocío Martinez Gallardo, Lynn Manson, David C Kluth
BACKGROUND: Current recommendations for ANCA-associated vasculitis (AAV) support its management within a dedicated clinical service. Therapies for AAV are imperfect with many patients failing to achieve disease control and others experiencing disease relapse. Plasma exchange (PEX) may be beneficial especially when the kidney is involved. METHODS: Within a new, dedicated service we retrospectively assessed, over a 6-year period, the benefits of PEX in two patient cohorts, discriminated by PEX treatment alone...
November 9, 2015: BMC Musculoskeletal Disorders
China Nagano, Yoshimitu Goto, Katuaki Kasahara, Yoshiyuki Kuroyanagi
BACKGROUND: Anti-glomerular basement membrane (GBM) antibody disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis caused by autoantibodies against the α3-chain of type IV collagen in the GBM. CASE PRESENTATION: An 8-year-old girl with hematuria and proteinuria due to anti-GBM nephritis was diagnosed with hematuria and proteinuria during a school urine screening program. Her blood pressure and serum creatinine levels were normal...
2015: BMC Nephrology
Boudhayan Das Munshi, Sarbani Sengupta, Abhijeet Sharan, Sarmishtha Mukhopadhyay, Bhaskar Ghosh, Angira Dasgupta, Rana Bhattacharyya
Pulmonary renal syndrome (PRS) is characterized by both diffuse alveolar haemorrhage and glomerulonephritis as pathological features. Several immunologic and non-immunologic mechanisms including anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus are commonly involved in the pathogenesis of the syndrome. We herein present a 60-year-old woman, non-smoker, who presented with fever, polyarthralgia, and the spreading of generalized purpuric rashes...
2015: Internal Medicine
Duvuru Geetha, Cees Kallenberg, John H Stone, Alan D Salama, Gerald B Appel, George Duna, Paul Brunetta, David Jayne
Granulomatosis with polyangiitis and microscopic polyangiitis are anti-neutrophil cytoplasmic antibody-associated vasculitides (AAVs) that are prone to cycles of remission and relapse. The introduction of cytotoxic therapy has changed the prognosis for these diseases from typically fatal to manageable chronic illnesses with a relapsing course. Despite improvements in outcomes, recurrence of disease and drug-related toxicity continue to produce significant morbidity and mortality. Better understanding of the pathogenesis of AAV and the mechanism of action of cyclophosphamide has led to investigation of therapies that target B cells...
February 2015: Journal of Nephrology
Takayuki Katsuyama, Ken-Ei Sada, Hirofumi Makino
Although a new classification algorithm for systemic vasculitides was proposed by Watts et al. and the Chapel Hill Consensus Conference (CHCC) was updated in 2012, there are currently no validated diagnostic criteria for systemic vasculitides. The Diagnostic and Classification Criteria for Vasculitis study (DCVAS) is a global study to develop and improve the diagnostic criteria for systemic vasculitides. The epidemiology of systemic vasculitides differs widely among countries. For example, in the case of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, patients with microscopic polyangiitis (MPA) and with positivity for MPO-ANCA are predominant in Asian countries, whereas patients with granulomatosis with polyangiitis (GPA) and with positivity for PR3-ANCA are predominant in northern Europe and the United States...
December 2014: Allergology International: Official Journal of the Japanese Society of Allergology
2015-10-01 21:27:04
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