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anca associated vasculitis

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https://www.readbyqxmd.com/read/28416516/semaphorin-4d-inhibits-neutrophil-activation-and-is-involved-in-the-pathogenesis-of-neutrophil-mediated-autoimmune-vasculitis
#1
Masayuki Nishide, Satoshi Nojima, Daisuke Ito, Hyota Takamatsu, Shohei Koyama, Sujin Kang, Tetsuya Kimura, Keiko Morimoto, Takashi Hosokawa, Yoshitomo Hayama, Yuhei Kinehara, Yasuhiro Kato, Takeshi Nakatani, Yoshimitsu Nakanishi, Takeshi Tsuda, Jeong Hoon Park, Toru Hirano, Yoshihito Shima, Masashi Narazaki, Eiichi Morii, Atsushi Kumanogoh
OBJECTIVES: Inappropriate activation of neutrophils plays a pathological role in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The aim of this study was to investigate the functions of semaphorin 4D (SEMA4D) in regulation of neutrophil activation, and its involvement in AAV pathogenesis. METHODS: Serum levels of soluble SEMA4D were evaluated by ELISA. Blood cell-surface expression of membrane SEMA4D was evaluated by flow cytometry. To determine the functional interactions between neutrophil membrane SEMA4D and endothelial plexin B2, wild-type and SEMA4D(-/-) mice neutrophils were cultured with an endothelial cell line (MS1) stained with SYTOX green, and subjected to neutrophil extracellular trap (NET) formation assays...
April 17, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28414152/rituximab-for-immunologic-renal-disease-what-the-nephrologist-needs-to-know
#2
REVIEW
Andreas Kronbichler, Martin Windpessl, Herwig Pieringer, David R W Jayne
Rituximab (RTX), a chimeric, monoclonal anti-CD20 antibody, is increasingly used in immune-mediated renal diseases. While licensed in the induction treatment of ANCA-associated vasculitis, it represents one of the most commonly prescribed off-label drugs. Much of the information regarding its safety has been drawn from experience in hematology and rheumatology. Ample evidence illustrates the safety of RTX, however, rare but serious adverse events have emerged that include progressive multifocal leucoencephalopathy and hepatitis B reactivation...
April 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#3
REVIEW
Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito
Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates...
April 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28406765/maintenance-treatment-of-anca-associated-vasculitides
#4
REVIEW
Loic Guillevin
ANCA-associated vasculitis(AAV)covers a group of systemic necrotising vasculitides characterised by inflammation of small vessels, sometimes with granuloma, and associated with autoantibodies to neutrophil cytoplasmic proteases (proteinase-3 or myeloperoxidase). Potentially lethal if not promptly diagnosed and treated, AAV in most patients can be induced into remission with the current treatment modalities. However, the risk of relapse remains high, necessitating prolonged immunosuppressive or immunomodulating maintenance therapy, whose optimal duration remains undetermined...
April 5, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28400446/randomized-trial-of-c5a-receptor-inhibitor-avacopan-in-anca-associated-vasculitis
#5
David R W Jayne, Annette N Bruchfeld, Lorraine Harper, Matthias Schaier, Michael C Venning, Patrick Hamilton, Volker Burst, Franziska Grundmann, Michel Jadoul, István Szombati, Vladimír Tesař, Mårten Segelmark, Antonia Potarca, Thomas J Schall, Pirow Bekker
Alternative C activation is involved in the pathogenesis of ANCA-associated vasculitis. However, glucocorticoids used as treatment contribute to the morbidity and mortality of vasculitis. We determined whether avacopan (CCX168), an orally administered, selective C5a receptor inhibitor, could replace oral glucocorticoids without compromising efficacy. In this randomized, placebo-controlled trial, adults with newly diagnosed or relapsing vasculitis received placebo plus prednisone starting at 60 mg daily (control group), avacopan (30 mg, twice daily) plus reduced-dose prednisone (20 mg daily), or avacopan (30 mg, twice daily) without prednisone...
April 11, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28397718/clinical-predictors-of-outcome-in-patients-with-anti-neutrophil-cytoplasmic-autoantibody-related-renal-vasculitis-experiences-from-a-single-center
#6
Lei Pu, Gui-Sen Li, Yu-Rong Zou, Ping Zhang, Li Wang
BACKGROUND: Primary anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with outcome. The purpose of this study was to investigate the clinical determinants of mortality of patients with AAV-related renal injury in the first 2 years after diagnosis in a single West Chinese center. METHODS: Demographic and laboratory parameters of 123 consecutive patients with AAV-related renal injury diagnosed in Renal Division and Institute of Nephrology, Sichuan Provincial People's Hospital between 2004 and 2012 were collected retrospectively...
April 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28397288/extensive-levamisole-induced-vasculitis
#7
C Desvignes, C Becquart, D Launay, L Terriou, P Patenotre, S Deheul, G Peytavin, N Dupin, E Delaporte, D Staumont-Sallé
Levamisole (an increasingly frequent contaminant of cocaine) can cause antineutrophil cytoplasmic antibody-associated vasculitis. Dermatologists should consider a diagnosis of cocaine/levamisole-associated cutaneous vasculopathy syndrome in cases of purpura of the ears and/or extensive retiform purpura in drug users. We report a case of particularly severe levamisole-induced necrotic purpura and immunological abnormalities in a 40-year-old woman.
April 10, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28397125/diffuse-alveolar-hemorrhage-in-autoimmune-diseases
#8
REVIEW
Marco Ulises Martínez-Martínez, David Alejandro Herrera-van Oostdam, Carlos Abud-Mendoza
PURPOSE OF REVIEW: The present paper establishes a narrative and analytical review of diffuse alveolar hemorrhage (DAH) in ANCA-associated vasculitis, systemic lupus erythematosus, and antiphospholipid syndrome. RECENT FINDINGS: Recent studies found a frequent association between DAH and infections and systemic lupus erythematosus and its associated factors. Biological therapies like rituximab have demonstrated benefit mainly in patients with ANCA-associated vasculitis...
May 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28391344/is-there-a-role-for-tnf%C3%AE-blockade-in-anca-associated-vasculitis-and-glomerulonephritis
#9
Stephen P McAdoo, Charles D Pusey
Tumour necrosis factor alpha (TNFα) is a cytokine that is pivotal in the inflammatory response. Blockade of TNFα has been shown to be effective in a number of human autoimmune diseases, including rheumatoid arthritis, raising the question of whether this approach may be effective in inflammatory kidney disease, such as ANCA-associated vasculitis (AAV). In AAV, there is considerable evidence for the role of TNFα in the pathophysiology of disease, including increased expression of TNFα mRNA in leucocytes and in renal tissue...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28391334/complement-activation-in-pauci-immune-necrotizing-and-crescentic-glomerulonephritis-results-of-a-proteomic-analysis
#10
Sanjeev Sethi, Ladan Zand, An S De Vriese, Ulrich Specks, Julie A Vrana, Siddak Kanwar, Paul Kurtin, Jason D Theis, Andrea Angioi, Lynn Cornell, Fernando C Fervenza
Background: Complement activation plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), although it remains unclear which pathway is activated. Whether pauci-immune necrotizing crescentic glomerulonephritis (pauci-immune GN) with negative ANCA serology is part of the spectrum of AAV or a different disease entity is essentially unknown. Methods: We used proteomic analysis to delineate the complement profile in a series of 13 kidney biopsies of patients with pauci-immune GN, with either proteinase 3 (PR3) (five patients) or myeloperoxidase (MPO) antibodies (four patients) or with consistently negative ANCA serology (four patients)...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28382598/patients-with-anca-associated-vasculitis-admitted-to-the-intensive-care-unit-with-acute-vasculitis-manifestations-a-retrospective-and-comparative-multicentric-study
#11
Julien Demiselle, Johann Auchabie, François Beloncle, Philippe Gatault, Steven Grangé, Damien Du Cheyron, Jean Dellamonica, Sonia Boyer, Dimitri Titeca Beauport, Lise Piquilloud, Julien Letheulle, Christophe Guitton, Nicolas Chudeau, Guillaume Geri, François Fourrier, René Robert, Emmanuel Guérot, Julie Boisramé-Helms, Pierre Galichon, Pierre-François Dequin, Alexandre Lautrette, Pierre-Edouard Bollaert, Ferhat Meziani, Loïc Guillevin, Nicolas Lerolle, Jean-François Augusto
PURPOSE: Data for ANCA-associated vasculitis (AAV) patients requiring intensive care are scarce. METHODS: We included 97 consecutive patients with acute AAV manifestations (new onset or relapsing disease), admitted to 18 intensive care units (ICUs) over a 10-year period (2002-2012). A group of 95 consecutive AAV patients with new onset or relapsing disease, admitted to two nephrology departments with acute vasculitis manifestations, constituted the control group...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28371513/early-outcomes-in-children-with-antineutrophil-cytoplasmic-antibody-anca-associated-vasculitis-aav
#12
Kimberly A Morishita, Lakshmi N Moorthy, Joanna M Lubieniecka, Marinka Twilt, Rae S M Yeung, Mary B Toth, Susan Shenoi, Goran Ristic, Susan M Nielson, Raashid A Luqmani, Suzanne C Li, Tzielan Lee, Erica F Lawson, Mikhail M Kostik, Marisa Klein-Gitelman, Adam M Huber, Aimee O Hersh, Dirk Foell, Melissa E Elder, Barbara A Eberhard, Paul Dancey, Sirirat Charuvanij, Susanne M Benseler, David A Cabral
Objective To characterize early disease course in childhood onset antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) and 12-month outcomes. Methods Eligible subjects were children diagnosed with GPA, MPA, EGPA, and ANCA-positive pauci-immune glomerulonephritis before their eighteenth birthday and entered into The Pediatric Vasculitis Initiative (PedVas) study. The primary outcome was remission (Pediatric Vasculitis Activity Score (PVAS) = 0 with corticosteroid dose (CS) <0.2mg/kg/day) at 12-months...
March 28, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28367219/alpha-1-antitrypsin-deficiency-presenting-with-mpo-anca-associated-vasculitis-and-aortic-dissection
#13
Bram M Voorzaat, Jan van Schaik, Stijn L P Crobach, Catharina S P van Rijswijk, Joris I Rotmans
The combination of alpha-1 antitrypsin (AAT) deficiency, ANCA-vasculitis, and aortic aneurysm has been rarely described in literature. We report an eventually fatal case in a 70-year-old patient who initially presented with giant cell arteritis and ANCA associated glomerulonephritis. Several years later, he presented with aortic dissection due to large vessel vasculitis, raising the suspicion of AAT deficiency, as two first-line relatives had chronic obstructive pulmonary disease, while they never smoked. This diagnosis was confirmed by AAT electrophoresis and immunohistochemistry on a temporal artery biopsy...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28361331/anca-associated-vasculitis-pathogenesis-a-commentary
#14
REVIEW
Eric J Gapud, Philip Seo, Brendan Antiochos
PURPOSE OF REVIEW: The ANCA-associated vasculitides are a group of small vessel vasculitides characterized by autoantibodies recognizing the neutrophil cytoplasmic antigens PR3 and MPO. We examine the current clinical and molecular immunology understanding of ANCA-associated vasculitides and discuss the current needs in our understanding of the pathogenic mechanisms of these rare diseases. RECENT FINDINGS: The majority of efforts to understand the pathogenesis of these diseases have focused on dissecting neutrophil biology because the neutrophil is the primary expressor of ANCA autoantigens...
April 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28356661/anti-glomerular-basement-membrane-disease-case-series-from-a-tertiary-center-in-north-india
#15
D Prabhakar, M Rathi, R Nada, R W Minz, V Kumar, H S Kohli, V Jha, K L Gupta
Anti-glomerular basement (anti-GBM) disease is an uncommon disorder with a bimodal age of presentation. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of anti-GBM disease from India. We conducted this prospective study to analyze the clinical presentation and outcomes of anti-GBM disease at a large tertiary care hospital in North India over 1½ years. Subjects with a biopsy proven anti-GBM disease (light microscopic examination showing crescents and immunofluorescence examination showing linear deposition of IgG) with or without positive anti-GBM antibodies in serum were included in the study and followed-up for at least 12 months...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28356090/anca-vasculitis-in-a-patient-with-alport-syndrome-a-difficult-diagnosis-but-a-treatable-disease
#16
Valentine Gillion, Michel Jadoul, Selda Aydin, Nathalie Godefroid
BACKGROUND: Alport syndrome and ANCA-associated vasculitis are both rare diseases. The co-existence of these two conditions has never been reported. There is no obvious pathogenic link between these two glomerular diseases. The management of this case highlights the importance of a systematic approach when investigating the unexpected unfavourable evolution of a known glomerulopathy. CASE PRESENTATION: A-17 year old caucasian boy with a genetically proven X-linked Alport syndrome presented with progressive dyspnea, fatigue and pallor...
March 29, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28355982/low-copy-numbers-of-fcgr3a-and-fcgr3b-associated-with-chinese-patients-with-sle-and-aasv
#17
Y Qi, X Zhou, D Bu, P Hou, J Lv, H Zhang
Low-affinity Fcγ receptors (FcγR) act as key mediators of the pathogenic effects of autoantibodies. In this study, we aimed to determine whether copy number variations (CNVs) in FCGR3A and FCGR3B were associated with systemic lupus nephritis (SLE) and ANCA-associated systemic vasculitis (AASV) in Chinese individuals. A total of 1118 individuals were enrolled, including 415 SLE patients, 139 AASV patients, and 564 healthy controls. FCGR3A and FCGR3B copy numbers (CNs) were determined by both a paralogue ratio test and TaqMan quantitative PCR assay...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28353556/granulomatosis-with-polyangiitis-presenting-with-diffuse-alveolar-hemorrhage-requiring-extracorporeal-membrane-oxygenation-with-rapid-multiorgan-relapse-a-case-report
#18
Jennifer Vanoli, Marta Riva, Beatrice Vergnano, Gabriele D'Andrea, Vincenzo L'Imperio, Maria Rosa Pozzi, Guido Grassi
RATIONALE: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitis affecting small- and medium-sized blood vessels, mostly involving lung and kidney. PATIENT CONCERNS: We report the case of a 33-year-old man that presented with acute respiratory distress syndrome caused by alveolar hemorrhage. DIAGNOSES: Aggressive GPA presenting with diffuse alveolar hemorrhage and multiorgan involvement...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28352023/anti-glomerular-basement-membrane-a-rare-cause-of-renal-failure-in-children
#19
Indira Agarwal, Ahmad Al-Ghitany
Anti-glomerular basement membrane (GBM) disease is a rare cause of acute renal failure and known to have bad prognosis regarding renal functions recovery and patient survival specially when diagnosed late and presents with severe renal failure that requires dialysis. We report a case of 11-year-old child with acute renal failure secondary to anti-GBM disease and associated with antineutrophil cytoplasmic antibody-positive vasculitis. He was treated with plasmapheresis, steroids, and cyclophosphamide with recovery of his kidney functions...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28348948/microscopic-polyangiitis-atypical-presentation-with-extensive-small-bowel-necrosis-diffuse-alveolar-hemorrhage-and-renal-failure
#20
Justin M Segraves, Vivek N Iyer
Microscopic polyangiitis is an uncommon systemic vasculitis of varying severity that is associated with myeloperoxidase (MPO) and perinuclear antineutrophil cytoplasmic (p-ANCA) antibodies. The most commonly affected organs are the lungs and kidneys. We report on a very unusual case of microscopic polyangiitis presenting with severe mesenteric ischemia in addition to diffuse alveolar hemorrhage and acute renal failure. The patient was initially diagnosed with acute pancreatitis at an outside facility given his severe abdominal pain and elevated pancreatic enzymes...
2017: Respiratory Medicine Case Reports
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