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ANCA glomerulonephritis

Gen Nakamura, Yuta Fukuchi, Noriyuki Homma
No abstract text is available yet for this article.
March 14, 2018: Clinical and Experimental Nephrology
Eli M Miloslavsky, John L Niles, Zachary S Wallace, Frank B Cortazar, Ana Fernandes, Karen Laliberte, John H Stone
OBJECTIVE: Therapeutic advances in ANCA-associated vasculitis (AAV) have improved patient survival, but mortality rates remain higher than the general population. Glucocorticoids contribute to AAV morbidity and mortality. We examined whether an 8-week glucocorticoid course in combination with rituximab (RTX) would induce disease remission in patients with AAV. METHODS: Patients with active AAV received an 8-week prednisone taper and RTX 375mg/m2 weekly for 4 weeks...
February 3, 2018: Seminars in Arthritis and Rheumatism
Bharat Kumar, Jennifer Strouse, Melissa Swee, Petar Lenert, Manish Suneja
INTRODUCTION: Hydralazine is an antihypertensive medication that has been associated with drug-induced lupus erythematosus (DIL) as well as ANCA-associated vasculitis (AAV). Although rare, early diagnosis is critical since drug cessation is the mainstay of therapy. This retrospective study aims to characterize the clinical, laboratory, and histopathologic features of this disease. METHODS: Once approval was obtained from the Institutional Review Board at the University of Iowa, all patients carrying a diagnosis of vasculitis (ICD9 code: 447...
January 12, 2018: Seminars in Arthritis and Rheumatism
Suheyla Apaydin
Therapeutic plasma aphresis (plasmapheresis) is one form of treatment that is frequently used in practice of Nephrology. Plasmapheresis is the most important part of the therapies for Goodpasture's syndrome and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis which are causes of rapidly progressive glomerulonephritis. The reason why the effectiveness of plasmapheresis therapy cannot be clearly demonstrated in renal involvement in these diseases is that it does not appear to be possible to recruit an adequate number of patients and plasmapheresis is not effective in advanced disease if early treatment is not initiated...
February 20, 2018: Transfusion and Apheresis Science
Sonja Krohn, Jasper F Nies, Sonja Kapffer, Tilman Schmidt, Jan-Hendrik Riedel, Anna Kaffke, Anett Peters, Alina Borchers, Oliver M Steinmetz, Christian F Krebs, Jan-Eric Turner, Silke R Brix, Hans-Joachim Paust, Rolf A K Stahl, Ulf Panzer
The IL-17 cytokine family and the cognate receptors thereof have a unique role in organ-specific autoimmunity. Most studies have focused on the founding member of the IL-17 family, IL-17A, as the central mediator of diseases. Indeed, although pathogenic functions have been ascribed to IL-17A and IL-17F in the context of immune-mediated glomerular diseases, the specific functions of the other IL-17 family members in immunity and inflammatory kidney diseases is largely unknown. Here, we report that compared with healthy controls, patients with acute Anti-neutrophil cytoplasmatic antibody (ANCA)-associated crescentic glomerulonephritis (GN) had significantly elevated serum levels of IL-17C (but not IL-17A, F, or E)...
February 26, 2018: Journal of the American Society of Nephrology: JASN
Gerald B Appel, Wai L Lau
As the population worldwide ages, the epidemic of kidney disease will also increase. Anti-neutrophil cytoplasmic antibodies (ANCA) positive rapidly progressive positive glomerulonephritis (RPGN) is the most common etiology for biopsied patients among the very elderly. Its pathological features and clinical course are well described, though there is still debate about the mechanism of injury involved in individual patients. From very ancient times, the cornerstone of treatment historically has been high-dose cyclophosphamide and a lengthy course of high-dose corticosteroids...
January 26, 2018: Blood Purification
Yoshihiro Aoki, Katsuhiko Kitazawa, Hironobu Kobayashi
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is rare in children and is characterised as necrotising vasculitis predominantly affecting small and medium-sized vessels. Propylthiouracil (PTU), an antithyroid drug, has been implicated in drug-induced AAV. In contrast, Kawasaki disease (KD) is a common systemic vasculitis, typically observed in children, which affects the medium-sized vessels, including the coronary arteries. An 11-year-old girl who developed AAV while receiving PTU therapy for Graves' disease is described...
February 19, 2018: Paediatrics and International Child Health
Silke R Brix, Mercedes Noriega, Elisabeth M Herden, Birgit Goldmann, Ulrike Langbehn, Martin Busch, Wolfram J Jabs, Oliver M Steinmetz, Ulf Panzer, Tobias B Huber, Rolf A K Stahl, Thorsten Wiech
AIMS: Renal involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis contributes to significant morbidity and mortality in patients. In chronic inflammation, B cells are recruited to the inflamed tissue and organised lymphoid structures have been described in several autoimmune diseases. Aim of this study was to correlate the lymphoid organisation in renal biopsies with renal outcome in ANCA-associated glomerulonephritis (GN). METHODS AND RESULTS: We investigated 112 renal biopsies from patients with newly diagnosed ANCA-associated necrotizing GN...
February 17, 2018: Histopathology
Y H Chen, X Liu, Z Z Liu, K Li, L Yang, H T Zhang, C H Zeng, F Xu, W X Hu
Objective: To explore the prognosis and its risk factors in anti-neutrophil cytoplasmic antibodies (ANCA)-associated glomerulonephritis (AAGN) patients who needed initial renal replacement therapy (RRT). Methods: One hundred patients [54 females, 46 males, with a median age of 54(41, 60) years] with biopsy-proven AAGN and requiring initial RRT between January 1996 and December 2016 in Nanjing Jinling Hospital were included. Intensive immunotherapy indicated that the patients received corticosteroids in combination with cyclophosphamide or mycophenolate mofetil, or immunoadsorption (IA) or double filtration plasmapheresis (DFPP)...
January 23, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
James Goya Heaf, Alastair Hansen, Gunnar Hellmund Laier
BACKGROUND: The association of increased cancer risk with glomerulonephritis (GN) is well known, but controversy exists concerning which types of GN are involved, and the size of the association. A national registry survey was performed to assess the size of this association, and the temporal relationship of cancer diagnosis to GN diagnosis. METHODS: All patients with biopsy-proven GN between 1985 and 2015 in Denmark were extracted from The Danish Renal Biopsy Registry and the National Pathology Data Bank...
February 2, 2018: BMC Nephrology
Ameni Jerbi, Sawsan Feki, Salma Ben Ameur, Hend Hachicha, Lamia Gargouri, Thouraya Kamoun, Abdelmajid Mahfoudh, Mongia Hachicha, Hatem Masmoudi
In adults, anti-neutrophil cytoplasmic antibodies (ANCA) are considered as serological markers of several diseases, especially vasculitis and glomerulonephritis. Since ANCA are rarely positive in children, few data about the clinical relevance of these auto-antibodies in pediatric population have been reported. Therefore, our study aims to describe the spectrum of disorders associated with positive ANCA in Tunisian children. This study had been carried out over a period of 12 years and a half. All patients under the age of 15 for whom ANCA screening was performed in our laboratory were included...
January 1, 2018: Annales de Biologie Clinique
Marion Haubitz
In patients with ANCA-associated vasculitis renal involvement is frequently seen and the severity of renal manifestation is very important for therapeutic strategies and prognosis. Clinically rapid loss of renal function, nephritic sediment and proteinuria in a non-nephrotic range are characterizing a focal segmental necrotizing pauci-immune glomerulonephritis with extrarenal proliferations. Induction treatment depends on the severity of manifestations. With a normal renal function methotrexate can be used in combination with steroids...
January 2018: Deutsche Medizinische Wochenschrift
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
Jonathan Dick, Poh-Yi Gan, A Richard Kitching, Stephen R Holdsworth
The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides are autoimmune diseases associated with significant morbidity and mortality. They often affect the kidney causing rapidly progressive glomerulonephritis. While signalling by complement anaphylatoxin C5a though the C5a receptor is important in this disease, the role of the anaphylatoxin C3a signalling via the C3a receptor (C3aR) is not known. Using two different murine models of anti-myeloperoxidase (MPO) glomerulonephritis, one mediated by passive transfer of anti-MPO antibodies, the other by cell-mediated immunity, we found that the C3aR did not alter histological disease severity...
2018: PloS One
Christian F Krebs, Ulf Panzer
Anti-neutrophil cytoplasmatic antibody (ANCA)-associated glomerulonephritis, anti-glomerular basement membrane (GBM) glomerulonephritis and lupus nephritis are the most common causes of rapid progressive glomerulonephritis (RPGN) in the Western world. These aggressive forms of autoimmune kidney diseases significantly contribute to end-stage renal disease and are associated with high morbidity and mortality. Moreover, patients show significant heterogeneity with respect to clinical outcome and response to therapy...
December 21, 2017: Journal of Autoimmunity
Jonathan Dick, Poh-Yi Gan, Sharon L Ford, Dragana Odobasic, Maliha A Alikhan, Sven H Loosen, Pam Hall, Clare L Westhorpe, Anqi Li, Joshua D Ooi, Trent M Woodruff, Charles R Mackay, A Richard Kitching, Michael J Hickey, Stephen R Holdsworth
The prospects for complement-targeted therapy in ANCA-associated vasculitis have been enhanced by a recent clinical trial in which C5a receptor 1 (C5aR1) inhibition safely replaced glucocorticoids in induction treatment. C5aR1 primes neutrophils for activation by anti-neutrophil cytoplasmic antibody (ANCA) and is therefore required in models of glomerulonephritis induced by anti-myeloperoxidase antibody. Although humoral and cellular autoimmunity play essential roles in ANCA-associated vasculitis, a role for C5aR1 in these responses has not been described...
December 11, 2017: Kidney International
Abhilash Koratala, Xu Zeng, Amir Kazory
IgA nephropathy, the most prevalent form of primary glomerular disease, usually portends a favourable outcome. Antineutrophil cytoplasmic autoantibodies (ANCAs) have been reported in association with IgA nephropathy in a small subset of patients, mostly presenting with rapidly progressive glomerulonephritis and necrotising crescentic lesions. Herein, we describe a case of IgA nephropathy, positive serum cytoplasmic and perinuclear ANCAs with anti-myeloperoxidase antibody, and preserved renal function without any histological evidence of necrotising or crescentic glomerulonephritis...
November 23, 2017: BMJ Case Reports
Emma E van Daalen, J Charles Jennette, Stephen P McAdoo, Charles D Pusey, Marco A Alba, Caroline J Poulton, Ron Wolterbeek, Tri Q Nguyen, Roel Goldschmeding, Bassam Alchi, Meryl Griffiths, Janak R de Zoysa, Beula Vincent, Jan A Bruijn, Ingeborg M Bajema
BACKGROUND AND OBJECTIVES: Large studies on long-term kidney outcome in patients with anti-glomerular basement membrane (anti-GBM) GN are lacking. This study aimed to identify clinical and histopathologic parameters that predict kidney outcome in these patients. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This retrospective analysis included a total of 123 patients with anti-GBM GN between 1986 and 2015 from six centers worldwide. Their kidney biopsy samples were classified according to the histopathologic classification for ANCA-associated GN...
January 6, 2018: Clinical Journal of the American Society of Nephrology: CJASN
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
Viviana Scollo, Luca Zanoli, Elisa Russo, Giulio Distefano, Francesco Rapisarda
It is well known that some disorders can cause concomitant kidney dysfunction with lung involvement. These syndromes, characterized by the simultaneous presence of intra-alveolar hemorrhage and acute glomerulonephritis, are caused by numerous and variable disorders. The most frequent are the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and Goodpasture syndrome. A quick discerning of the underlying causes and initiation of adequate treatment is crucial to prevent acute respiratory failure and irreversible loss of renal function...
2017: Clinical Medicine Insights. Case Reports
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