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https://www.readbyqxmd.com/read/28537941/pathophysiology-of-anca-associated-vasculitis
#1
Turki Al-Hussain, Maged H Hussein, Walter Conca, Hadeel Al Mana, Mohammed Akhtar
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is characterized as inflammation of small-sized to medium-sized blood vessels and encompasses several clinicopathologic entities including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited ANCA-associated vasculitis. Over the past several decades, significant progress has been made in understanding the pathophysiology of ANCA-associated vasculitis. Although neutrophils contain a multitude of granular proteins, clinically significant autoantibodies are only recognized against myeloperoxidase and proteinase 3, both of which are present in the azurophilic granules...
May 22, 2017: Advances in Anatomic Pathology
https://www.readbyqxmd.com/read/28515156/anti-glomerular-basement-membrane-disease
#2
Stephen P McAdoo, Charles D Pusey
Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is an archetypic autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well characterized autoantigen expressed in the basement membranes of these organs, although the inciting events that induce the autoimmune response are not fully understood. The recent confirmation of spatial and temporal clustering of cases suggests that environmental factors, including infection, may trigger disease in genetically susceptible individuals...
May 17, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28509135/renal-limited-vasculitis-with-elevated-levels-of-multiple-antibodies
#3
Noriaki Sato, Hideki Yokoi, Hirotaka Imamaki, Eiichiro Uchino, Kaoru Sakai, Takeshi Matsubara, Tatsuo Tsukamoto, Sachiko Minamiguchi, Motoko Yanagita
Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509134/two-cases-of-atypical-hemolytic-uremic-syndrome-ahus-and-eosinophilic-granulomatosis-with-polyangiitis-egpa-a-possible-relationship
#4
Mercedes Cao, Tamara Ferreiro, Bruna N Leite, Francisco Pita, Luis Bolaños, Francisco Valdés, Angel Alonso, Eduardo Vázquez, Juan Mosquera, María Trigás, Santiago Rodríguez
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal failure. It is related to genetic mutations of the alternative complement pathway and is difficult to differentiate from other prothrombotic microangiopathies. Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell apoptosis and necrosis and therefore, vasculitis...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508972/development-of-intracerebral-hemorrhage-in-the-short-term-clinical-course-of-a-patient-with-microscopic-polyangiitis-without-neurological-symptoms-at-diagnosis-an-autopsy-case
#5
Yoshia Miyawaki, Takayuki Katsuyama, Ken-Ei Sada, Kohei Taniguchi, Yuki Kakio, Jun Wada
A 77-year-old man with high-grade fever, progressive renal dysfunction, high serum level of C-reactive protein and positive serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) was diagnosed with microscopic polyangiitis with rapidly progressive glomerulonephritis, and remission induction treatment with glucocorticoids and intravenous cyclophosphamide was initiated. Although his general condition improved in a short time, intracerebral hemorrhage occurred 12 days after the initiation of treatment and emergent hematoma evacuation was performed...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508096/-off-label-biologic-therapy-of-anca-associated-and-non-anca-associated-small-vessel-vasculitis-efficacy-and-safety-analysis-of-a-national-registry-graid2
#6
N Venhoff, F Proft, H Schulze-Koops, J Holle, R E Voll, C Iking-Konert, A M Jacobi, J Henes, L Unger, O Kneitz, T Dörner, J Thiel
OBJECTIVE: To evaluate the clinical efficacy and safety of off-label biological therapies in patients with ANCA-associated vasculitis (AAV) and non-ANCA-associated small-vessel vasculitis (nAAV) in clinical practice. METHODS: The German Registry in Autoimmune Diseases 2 (GRAID2) is a national, retrospective, non-interventional, multicentre observational study (August 2006 until December 2013) on patients with autoimmune diseases refractory to standard immunosuppressive therapy treated with off-label biologicals...
May 15, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28507487/demographic-characteristics-aetiology-and-assessment-of-treatment-options-in-leukocytoclastic-vasculitis
#7
Alkim Unal Cakiter, Ozlem Su Kucuk, Dilek Biyik Ozkaya, Bugce Topukcu, Nahide Onsun
INTRODUCTION: Vasculitides are a heterogeneous group of diseases characterized by inflammation of the blood vessel walls. Etiological factors include infections, drugs, connective tissue diseases, and malignancies. AIM: To examine the demographic characteristics, etiological factors, and treatment options in 75 patients with leukocytoclastic vasculitis. MATERIAL AND METHODS: The study included 75 patients diagnosed with leukocytoclastic vasculitis at our clinic...
April 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28506760/patients-double-seropositive-for-anca-and-anti-gbm-antibodies-have-varied-renal-survival-frequency-of-relapse-and-outcomes-compared-to-single-seropositive-patients
#8
Stephen P McAdoo, Anisha Tanna, Zdenka Hrušková, Lisa Holm, Maria Weiner, Nishkantha Arulkumaran, Amy Kang, Veronika Satrapová, Jeremy Levy, Sophie Ohlsson, Vladimir Tesar, Mårten Segelmark, Charles D Pusey
Co-presentation with both ANCA and anti-GBM antibodies is thought to be relatively rare. Current studies of such 'double-positive' cases report small numbers and variable outcomes. To study this further we retrospectively analyzed clinical features and long-term outcomes of a large cohort of 568 contemporary patients with ANCA-associated vasculitis, 41 patients with anti-GBM disease, and 37 double-positive patients with ANCA and anti-GBM disease from four European centers. Double-positive patients shared characteristics of ANCA-associated vasculitis (AAV), such as older age distribution and longer symptom duration before diagnosis, and features of anti-GBM disease, such as severe renal disease and high frequency of lung hemorrhage at presentation...
May 12, 2017: Kidney International
https://www.readbyqxmd.com/read/28502640/a-cohort-study-reveals-myocarditis-to-be-a-rare-and-life-threatening-presentation-of-large-vessel-vasculitis
#9
Katie Bechman, Deepa Gopalan, Petros Nihoyannopoulos, Justin C Mason
BACKGROUND: The predominant forms of adult large vessel vasculitis (LVV) are giant cell arteritis (GCA) and Takayasu arteritis (TA). Cardiac involvement in LVV is a cause of morbidity and mortality, particularly in TA. Cardiac failure is most commonly secondary to uncontrolled arterial hypertension or myocardial ischaemia. Pulmonary hypertension and aortic valve incompetence following ascending aortic dilatation represent other serious cardiovascular complications. However, cardiac failure as a consequence of myocarditis is rarely reported, principally in single case reports or in autopsy studies...
April 4, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28491854/mpo-anca-associated-necrotizing-glomerulonephritis-in-rheumatoid-arthritis-a-case-report-and-review-of-literature
#10
Mário Góis, Ana Messias, Dulce Carvalho, Fernanda Carvalho, Helena Sousa, João Sousa, Fernando Nolasco
BACKGROUND: Renal involvement in rheumatoid arthritis (RA) is common and has a negative impact on patient survival. Only few cases have been reported of necrotizing glomerulonephritis (GN) associated with myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) in patients with RA. CASE PRESENTATION: We report a patient with RA who developed a necrotizing GN associated with ANCA-MPO, treated with rituximab (RTX). A 55-year-old man with a 27-year history of RA under secukinumab was referred to our nephrology clinic with worsening renal function associated with microhematuria and proteinuria...
March 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28476172/anca-associated-vasculitis-in-childhood-recent-advances
#11
REVIEW
Marta Calatroni, Elena Oliva, Davide Gianfreda, Gina Gregorini, Marco Allinovi, Giuseppe A Ramirez, Enrica P Bozzolo, Sara Monti, Claudia Bracaglia, Giulia Marucci, Monica Bodria, Renato A Sinico, Federico Pieruzzi, Gabriella Moroni, Serena Pastore, Giacomo Emmi, Pasquale Esposito, Mariagrazia Catanoso, Giancarlo Barbano, Alice Bonanni, Augusto Vaglio
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome...
May 5, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28454112/the-challenge-of-treating-hepatitis-c-virus-associated-cryoglobulinemic-vasculitis-in-the-era-of-anti-cd20-monoclonal-antibodies-and-direct-antiviral-agents
#12
REVIEW
Dario Roccatello, Savino Sciascia, Daniela Rossi, Laura Solfietti, Roberta Fenoglio, Elisa Menegatti, Simone Baldovino
Mixed cryoglobulinemia syndrome (MC) is a systemic vasculitis involving kidneys, joints, skin, and peripheral nerves. While many autoimmune, lymphoproliferative, and neoplastic disorders have been associated with this disorder, hepatitis C virus (HCV) is known to be the etiologic agent in the majority of patients. Therefore, clinical research has focused on anti-viral drugs and, more recently, on the new, highly potent Direct-acting Antiviral Agents (DAAs). These drugs assure sustained virologic response (SVR) rates >90%...
April 9, 2017: Oncotarget
https://www.readbyqxmd.com/read/28416004/clinical-study-of-children-with-takayasu-arteritis-a-retrospective-study-from-a-single-center-in-china
#13
Ye Feng, Xuemei Tang, Mingyue Liu, Juan Zhou, Xiaodong Zhao, Qiu Li
BACKGROUND: Delayed diagnosis of childhood Takayasu arteritis (TA) is common due to its atypical symptoms. The objective of the present study was to summarize the clinical features of childhood TA to raise awareness and improve management. METHODS: Eleven children diagnosed with TA at our hospital were enrolled. Clinical information, diagnosis, treatment, and outcome were then examined retrospectively. The Pediatric Vasculitis Activity Score (PVAS) and the Indian Takayasu Clinical Activity Score (ITAS2010) were used to assess disease activity...
April 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28414152/rituximab-for-immunologic-renal-disease-what-the-nephrologist-needs-to-know
#14
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/28397718/clinical-predictors-of-outcome-in-patients-with-anti-neutrophil-cytoplasmic-autoantibody-related-renal-vasculitis-experiences-from-a-single-center
#15
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/28391344/is-there-a-role-for-tnf%C3%AE-blockade-in-anca-associated-vasculitis-and-glomerulonephritis
#16
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/28382598/patients-with-anca-associated-vasculitis-admitted-to-the-intensive-care-unit-with-acute-vasculitis-manifestations-a-retrospective-and-comparative-multicentric-study
#17
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/28374407/cutaneous-manifestations-of-diabetes-mellitus-a-review
#18
REVIEW
Ana Luiza Lima, Tanja Illing, Sibylle Schliemann, Peter Elsner
Diabetes mellitus is a widespread endocrine disease with severe impact on health systems worldwide. Increased serum glucose causes damage to a wide range of cell types, including endothelial cells, neurons, and renal cells, but also keratinocytes and fibroblasts. Skin disorders can be found in about one third of all people with diabetes and frequently occur before the diagnosis, thus playing an important role in the initial recognition of underlying disease. Noninfectious as well as infectious diseases have been described as dermatologic manifestations of diabetes mellitus...
April 3, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28356675/thrombotic-microangiopathy-due-to-viperidae-bite-two-case-reports
#19
T Dineshkumar, J Dhanapriya, R Sakthirajan, K Thirumalvalavan, A A Kurien, T Balasubramaniyan, N Gopalakrishnan
Snake bite is mainly an occupational hazard and causes serious health problems in rural India. Acute kidney injury (AKI) occurs in 5-30% cases. Renal pathologic findings include acute tubular necrosis, cortical necrosis, interstitial nephritis, glomerulonephritis, and vasculitis. Thrombotic microangiopathy (TMA) occurrence after a snake bite is reported rarely. Here, we present two patients who developed TMA after viper bite treated with hemodialysis and plasmapheresis. Renal biopsy showed fibrin thrombi in glomeruli and arterioles with cortical necrosis...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28356661/anti-glomerular-basement-membrane-disease-case-series-from-a-tertiary-center-in-north-india
#20
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
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