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ANCA glomerulonephritis

Anne M Kouri, Sharon P Andreoli
BACKGROUND: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a small- and medium-sized vasculitis classically seen in adult patients, with peak onset near the fifth to seventh decade of life. There is little data on ANCA-associated vasculitis in pediatric patients, and most studies have limited follow-up. METHODS: This is a retrospective chart review of 22 patients with ANCA-positive glomerulonephritis in a single institution from 1991 to 2013...
September 27, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Vincent Cottin, Elisabeth Bel, Paolo Bottero, Klaus Dalhoff, Marc Humbert, Romain Lazor, Renato A Sinico, Pasupathy Sivasothy, Michael E Wechsler, Matthieu Groh, Sylvain Marchand-Adam, Chahéra Khouatra, Benoit Wallaert, Camille Taillé, Philippe Delaval, Jacques Cadranel, Philippe Bonniaud, Grégoire Prévot, Sandrine Hirschi, Anne Gondouin, Bertrand Dunogué, Gérard Chatté, Christophe Briault, Christian Pagnoux, David Jayne, Loïc Guillevin, Jean-François Cordier
OBJECTIVE: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. METHODS: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. RESULTS: The study population included 157 patients (mean age 49...
September 23, 2016: Autoimmunity Reviews
Rose M Ayoob, Andrew L Schwaderer
The most common acute glomerulonephritis in children is poststreptococcal glomerulonephritis (PSGN) usually occurring between 3 and 12 years old. Hypertension and gross hematuria are common presenting symptoms. Most PSGN patients do not experience complications, but rapidly progressive glomerulonephritis and hypertensive encephalopathy have been reported. This paper reports 17 patients seen in 1 year for PSGN including 4 with atypical PSGN, at a pediatric tertiary care center. Seventeen children (11 males), mean age of 8 years, were analyzed...
2016: International Journal of Nephrology
Manabu Tashiro, Yoshie Sasatomi, Renya Watanabe, Maho Watanabe, Katsuhisa Miyake, Yasuhiro Abe, Tetsuhiko Yasuno, Kenji Ito, Naoko Ueki, Aki Hamauchi, Ritsuya Noda, Satoshi Hisano, Hitoshi Nakashima
BACKGROUND: It is widely accepted that tubulointerstitial injury (TII) is caused by glomerular injury (GI) in glomerular diseases. Glomerular endocapillary inflammation may result in crescent formation and exuded protein leakage, which may induce TII in antineutrophil cytoplasmic antibody-associated glomerulonephritis (ANCAGN). However, some reports have indicated a glomerulonephritis-independent mechanism of TII in ANCAGN. The aim of this study was to determine the principle cytokines correlated with TII severity and to elucidate a characteristic mechanism for TII in ANCAGN...
October 2016: Clinical Nephrology
Huifang Liu, Jiachuan Xiong, Jun Zhang, Ying Zhang, Ling Nie, Yiqin Wang, Jinghong Zhao
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a life-threatening condition that causes renal failure. Multiple myeloma (MM) is a malignant proliferation of monoclonal plasma cells in the blood that can also cause renal failure. The two diseases have high morbidity and mortality rates in the elderly, with a poor prognosis. A 64-year-old female presented to Xinqiao Hospital (Chonqing, China) with fatigue and a poor appetite that had been apparent for 6 weeks. Laboratory tests revealed a serum creatinine level of 10...
September 2016: Oncology Letters
Yuichi Saito, Sho Okada, Nobusada Funabashi, Yoshio Kobayashi
A 59-year-old woman with a history of bronchial asthma presented with a prolonged fever and eosinophilia. There was transient proteinuria and troponin level was elevated. Antineutrophil cytoplasmic antibody was negative and she did not fulfil criteria for eosinophilic granulomatosis with polyangitis (EGPA). Echocardiography showed a large apical mass in the left ventricle, but there was no systolic dysfunction, local asynergy or ventricular remodelling. On MRI, apical mass was compatible with a thrombus and endocardial region was diffusely damaged...
2016: BMJ Case Reports
Shuichi Asano, Shige Mizuno, Shotaro Okachi, Hiromichi Aso, Keiko Wakahara, Naozumi Hashimoto, Satoru Ito, Yohei Kozaki, Takayuki Katsuno, Shoichi Maruyama, Yoshinori Hasegawa
A 73-year-old woman was diagnosed with pulmonary Mycobacterium avium complex (MAC) infection and received no treatment. Disease progression was evident one year later with the development of myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA) titers and systemic symptoms of a fever, polyarthritis, purpura, and rapidly progressive glomerulonephritis. Her symptoms did not improve with antibiotic treatment. A renal biopsy revealed crescentic glomerulonephritis with immunodeposition. According to these findings, she was diagnosed with ANCA-associated vasculitis (AAV) superimposed on infection-related glomerulonephritis (IRGN)...
2016: Internal Medicine
Manfred Relle, Bernd Föhr, Federica Fasola, Andreas Schwarting
Granulomatosis with polyangiitis (GPA) is a severe autoimmune disease and one of the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although its etiology and pathophysiology are still widely unknown, it is accepted that infections, environmental factors, epigenetic modifications, and a genetic predisposition provide the basis for this systemic disorder. GPA typically evolves into two phases: an initial phase characterized by ear, nose and throat (ENT) manifestations, such as chronic sinusitis and otitis, ulceration of the oral cavity and pharynx, as well as pulmonary nodules and a severe generalized phase, defined by the occurrence of rapidly progressive glomerulonephritis, pulmonary hemorrhage, and arthritis...
August 21, 2016: Molecular and Cellular Probes
Sophia Lionaki, John N Boletis
BACKGROUND: Pauci-immune glomerulonephritis is the most common cause of aggressive glomerulonephritis and occurs as a renal-limited disease or as a component of systemic necrotizing small-vessel vasculitis. It is characterized by paucity of staining for immunoglobulins, by immunofluorescence along with fibrinoid necrosis and crescent formation by light microscopy, while the vast majority of patients have anti-neutrophil cytoplasmic antibodies (ANCA) in their circulation, which also participate in the pathogenesis of the disease...
March 2016: Kidney Diseases
Zhi-Ying Li, Tian-Tian Ma, Min Chen, Ming-Hui Zhao
BACKGROUND: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Myeloperoxidase (MPO) and proteinase 3 (PR3) are the main antigens for ANCA. AAV is a common multisystem autoimmune disease and most of the studies on AAV have been conducted in Western countries. Nowadays in China many efforts are made to investigate this disease. SUMMARY: This review highlights the progress in the prevalence, management and outcomes of AAV in Chinese patients...
March 2016: Kidney Diseases
Hong Xiao, Peiqi Hu, Ronald J Falk, J Charles Jennette
BACKGROUND: Antineutrophil cytoplasmic autoantibodies (ANCA) are associated with a spectrum of necrotizing vasculitis including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited necrotizing and crescentic glomerulonephritis. Clinical observations and in vitro and in vivo experimental evidence strongly indicate that ANCA are pathogenic. SUMMARY: The etiology and pathogenesis of ANCA-associated vasculitis (AAV) are multifactorial, with contributions from genetic factors, environmental exposures, infections, characteristics of the innate and adaptive immune system, and the intensity and duration of the injury...
March 2016: Kidney Diseases
Duriye Deren Oygar, Yonca Morris
Behçet's disease (BD) is a multisystem disorder of unknown etiology. Renal involvement in Behçet's disease is not frequent. Pauciimmune necrotizing and crescentic glomerulonephritis is associated with antineutrophil cytoplasmic antibodies (ANCA) which are believed to activate neutrophils and cause vasculitis, especially if there is a concurrent synergistic inflammatory process. In this case report, we describe a patient diagnosed with BD who also developed pauciimmune necrotizing and crescentic glomerulonephritis...
2016: Internal Medicine
Supraja Yeturi, Mary Cronin, Adam Robin, Campbell Lorna, Ann K Rosenthal
Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren's syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus...
2016: Case Reports in Rheumatology
Ryo Koda, Katsuhiro Nagahori, Atsushi Kitazawa, Yuji Imanishi, Atsunori Yoshino, Shinya Kawamoto, Yoshihiko Ueda, Tetsuro Takeda
A 77-year-old man presented with a fever, non-productive cough, and edema formation. A laboratory analysis showed an elevated creatinine level (2.5 mg/dL), a high titer of myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) (99 U/mL), positive reaction for antinuclear antibody (×320), hematuria, and massive proteinuria (3.33 g/day). A renal biopsy revealed crescentic and necrotizing glomerulonephritis (GN) with membranoproliferative GN features [double contour appearance of the glomerular basement membrane, granular deposition of immunoglobulin (Ig) G, IgM, and C3 along the capillary wall, subendothelial and subepithelial deposits with mesangial interposition]...
2016: Internal Medicine
Yinghua Chen, Yuemei Ding, Zhengzhao Liu, Haitao Zhang, Zhihong Liu, Weixin Hu
BACKGROUND: The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic autoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have not been studied. METHODS: Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy. Clinic-pathological features and outcomes were retrospectively analyzed. RESULTS: The most common initial symptom of EGPA was asthma (57...
July 26, 2016: BMC Nephrology
Kiran P Sathe, Kumud P Mehta
The simultaneous presence of multiple immune-mediated diseases in a single host is rare. The implications of such coexistence relating to the disease pathogenesis and treatment are not well understood. We describe two cases of renal failure with immune-mediated overlap conditions. We believe, this is the first reported case of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis coexisting with atypical hemolytic uremic syndrome (HUS). Two boys aged eight years (Case 1) and 10 years (Case 2) presenting with renal failure secondary to glomerulonephritis are described...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
Mitsutoshi Shindo, Susumu Ookawara, Kiyonori Ito, Taisuke Kitano, Sawako Goto, Haruhisa Miyazawa, Yuichiro Ueda, Yoshio Kaku, Keiji Hirai, Taro Hoshino, Honami Mori, Izumi Yoshida, Kaoru Tabei, Yoshiyuki Morishita
A 68-year-old Japanese man was diagnosed with left otitis media with effusion and left uveitis more than 5 months before admission. He was urgently admitted to our hospital for progressive deterioration of his renal function [serum creatinine(Cr) 7.59 mg/dL] with proteinuria and urinary red blood cell casts, inflammation, and anemia. Additionally, his serum proteinase 3 antinuclear antibody (PR3-ANCA) level, determined by using the chemiluminescence enzyme immunoassay method, had increased to more than 3,500 U/mL...
2016: Nihon Jinzo Gakkai Shi
In Hee Lee, Seong-Kyu Kim, Min-Kyung Kim
Sjögren's syndrome is an autoimmune disease that primarily affects exocrine glands. Renal involvement of Sjögren's syndrome may lead to tubulointerstitial disease, whereas secondary glomerulopathies such as anti-neutrophil cytoplasmic antibody (ANCA)-related pauci-immune crescentic glomerulonephritis are rarely observed. In addition, crescent glomerulonephritis that is simultaneously positive for both myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA has never been reported in Sjögren's syndrome. Here, we report a case of pauci-immune crescentic glomerulonephritis exhibiting positivity for both MPO- and PR3-ANCAs in a patient with primary Sjögren's syndrome...
September 2016: Rheumatology International
Adrian Schreiber, Claudia Eulenberg-Gustavus, Astrid Bergmann, Uwe Jerke, Ralph Kettritz
ANCA to either PR3 or MPO are found in patients with necrotizing vasculitis and glomerulonephritis. ANCA binding to their target antigens on neutrophils and subsequent neutrophil activation are pivotal disease mechanisms that lead to vascular inflammation and necrosis. ANCA interaction with PR3 is more complex than with MPO as the neutrophil-specific CD177 receptor is involved in PR3 surface expression and PR3-ANCA-induced neutrophil activation. Modeling human disease is important to clinical research. Highly successful mouse models of MPO-ANCA vasculitis exist; however, recapitulating PR3-ANCA vasculitis has not been successful...
June 30, 2016: Journal of Leukocyte Biology
Kanupriya Mathur, Aditya Saini, Tonjeh Bah, Pavan Katikaneni
A 45-year-old Caucasian man presented to the hospital with a 3-month history of fatigue, bilateral upper and lower limb paresthesias and gradually worsening ascending paralysis. A few weeks later, he developed acute renal failure requiring haemodialysis. Investigations revealed presence of myeloperoxidase (MPO) perinuclear antineutrophil cytoplasmic antibodies (ANCA). Renal biopsy was conclusive for rapidly progressive glomerulonephritis with crescents. Treatment for ANCA positive vasculitis was initiated with pulsed steroids, cyclophosphamide and plasmapheresis...
2016: BMJ Case Reports
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