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Anti neutrophil cytoplasmic antibody

Mitsuhiro Akiyama, Tsutomu Takeuchi
IgG4-related disease is a heterogeneous immune-mediated fibroinflammatory condition that can affect every single organ. This disease is more prevalent in the elderly (the mean age of patients is above 60 years) and the prevalence rate is estimated to be over 4.6 per 100,000 population. Before making a diagnosis, the exclusion of malignancies, lymphoma, anti-neutrophil cytoplasmic antibody-associated vasculitis, multicentric Castleman disease, and other mimickers is crucial for appropriate treatment. Broad management guidelines have been published emphasizing the need for prompt treatment and the use of glucocorticoids as first-line drug therapy for induction of remission...
March 15, 2018: Drugs & Aging
Supaporn Suwanchote, Muanpetch Rachayon, Pongsawat Rodsaward, Jongkonnee Wongpiyabovorn, Tawatchai Deekajorndech, Helen L Wright, Steven W Edwards, Michael W Beresford, Pawinee Rerknimitr, Direkrit Chiewchengchol
Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies that cause systemic vascular inflammation by binding to target antigens of neutrophils. These autoantibodies can be found in serum from patients with systemic small-vessel vasculitis and they are considered as a biomarker for ANCA-associated vasculitis (AAV). A conventional screening test to detect ANCA in the serum is indirect immunofluorescence study, and subsequently confirmed by enzyme-linked immunosorbent assay. A positive staining of ANCA can be classified into three main categories based on the staining patterns: cytoplasmic, perinuclear, and atypical...
March 10, 2018: Clinical Rheumatology
H Marco, J Draibe, J Villacorta, L F Quintana, N Martin, R Garcia-Osuna, C Cabre, M A Martín-Gómez, A Balius, A Saurina, M Picazo, I Gich-Saladich, M Navarro-Díaz, M Praga, T Cavero, J Ballarin, M M Díaz-Encarnación
The classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains controversial. The main objective of this study was to define the respective values of ANCA serotype-based classification, clinicopathological classification, and histopathological classification in predicting patient and renal outcomes in a Spanish cohort of patients with ANCA with specificity for myeloperoxidase, MPO-ANCA, versus ANCA with specificity for proteinase 3, PR3-ANCA. Two hundred and forty-five patients with ANCA-AAV and biopsy-proven renal involvement diagnosed between 2000 and 2104 were recruited in 12 nephrology services...
March 9, 2018: Clinical Rheumatology
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
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
Zdenka Hruskova, Vladimir Tesar
BACKGROUND: Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid deterioration of renal function and by extracapillary proliferation in >50% of glomeruli. The most common type of RPGN is "pauci-immune" glomerulonephritis caused by anti-neutrophil cytoplasmic antibodies-associated vasculitis (AAV). SUMMARY: The incidence of AAV increases with age and pauci-immune glomerulonephritis is the most common diagnosis found in renal biopsies in the elderly population...
January 26, 2018: Blood Purification
Stephen P McAdoo, Nicholas Medjeral-Thomas, Seerapani Gopaluni, Anisha Tanna, Nicholas Mansfield, Jack Galliford, Megan Griffith, Jeremy Levy, Thomas D Cairns, David Jayne, Alan D Salama, Charles D Pusey
Background: Current guidelines advise that rituximab or cyclophosphamide should be used for the treatment of organ-threatening disease in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), although few studies have examined the efficacy and safety of these agents in combination. Methods: We conducted a single-centre cohort study of 66 patients treated with a combination of oral corticosteroids, rituximab and low-dose pulsed intravenous cyclophosphamide followed by a maintenance regimen of azathioprine and tapered steroid for the treatment of biopsy-proven renal involvement in AAV...
February 14, 2018: Nephrology, Dialysis, Transplantation
Mara Felicetti, Diego Cazzador, Roberto Padoan, Alfonso Luca Pendolino, Chiara Faccioli, Ennio Nardello, Alvise Berti, Marina Silvestrini, Giuseppe Paolazzi, Giuliano Brunori, Elisabetta Zanoletti, Enzo Emanuelli, Alessandro Martini, Franco Schiavon
Ear, nose and throat (ENT) manifestations in granulomatosis with polyangiitis (GPA) represent the most frequent symptoms at disease onset. The aim of the study was to analyse ENT involvement at diagnosis, as well as how it could influence relapse rate, mortality and disease severity. A retrospective non-controlled cohort study was performed including all consecutive diagnosed GPA from 1996 to 2016 in two rheumatology centres of Northern Italy, focusing particularly on ENT presenting signs and symptoms at baseline...
February 20, 2018: Clinical Rheumatology
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
Yosra Selmi, Rania Kheder-Elfekih, Hela Jebali, Lilia Ben Fatma, Wided Smaoui, Madiha Krid, Soumaya Beji, Lamia Rais, Mohamed Karim Zouaghi
Various adverse reactions may occur after intravesical bacillus Calmette-Guérin (BCG) therapy. Although the virulence of attenuated BCG is low, serious complications such as bacterial cystitis, bladder contractures, granulomatous prostatitis, epididymitis, orchitis, and systemic reactions such as fever and malaise have been described. Disseminated granulomatosis such as hepatitis and pneumonitis have also been described, but are rare. We report here the case of a 67-year-old patient who presented with renal granulomatosis with polyangiitis following intravesical BCG therapy for superficial bladder tumor...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
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
Duvuru Geetha, Sam Kant
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) has the propensity to acquire a devastating disease course. Despite the advances in therapeutics, a significant proportion of patients progress to end-stage renal disease (ESRD). Renal transplantation is being increasingly employed in this population, with gradual improvement in outcomes over the years, however, recurrence of disease requires constant surveillance and is associated with graft failure. Areas covered: A structured literature search in PubMed and Medline and abstracts of international conferences was performed to identify cases and cohorts of AAV patients who had undergone renal transplantation for ESRD...
February 13, 2018: Expert Review of Clinical Immunology
Toshiki Yamamoto, Hitomi Ryuzaki, Shun Kobayashi, Shu Ohshiro, Masahiro Ogawa, Naohide Tanaka, Takuji Gotoda, Mitsuhiko Moriyama, Noriko Kinukawa, Masahiko Sugitani, Kenji Notohara
The patient was a 72-year-old woman whose alkaline phosphatase levels had been elevated since she was 56 years old. Liver biopsies obtained when the patient was 64 and 66 years of age led to a suspicion of cholangitis caused by vasculitis. Furthermore, proteinase-3 anti-neutrophil cytoplasmic antibody positivity led to a suspicion of granulomatosis with polyangiitis, but subjective symptoms and disorders in other organs were absent, so this suspicion was not confirmed. Cholangitis caused by vasculitis rarely occurs without vasculitis in other organs...
February 9, 2018: Internal Medicine
B Sayyaf Dezfuli, M Manera, G Bosi, P Merella, J A DePasquale, L Giari
This investigation aims to fill gaps in our understanding of the intestinal immune cells of elasmobranchs. Whole digestive tracts of fifteen thornback ray Raja clavata were provided by a trawl fleet from the Gulf of Asinara (Sardinia, western Mediterranean Sea). Histochemical, immunohistochemical and ultrastructural observations were conducted on the spiral intestine. Three types of granular cells were identified; type I in epithelium, types II and III in lamina propria-submucosa, with each of them containing cytoplasmic granules with different ultrastructural characteristics...
February 9, 2018: Fish & Shellfish Immunology
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]
Elena Csernok, Juliane Mahrhold, Bernhard Hellmich
The current practice for detection of anti-neutrophil cytoplasm antibodies (ANCA) directed against proteinase 3 (PR3) and myeloperoxidase (MPO) has been screening by indirect immunofluorescence (IIF) followed by an antigen specific tests for PR3- and MPO-ANCA. However, ANCA diagnostics have undergone many technical developments that have affected the 1999 international consensus recommendations, and lead to a revision of the existing ANCA detection strategy. Recent European multicentre studies have compared the diagnostic performance of various ANCA detection methods and demonstrated that PR3- and MPO-ANCA immunoassays yielded the highest diagnostic accuracy...
January 27, 2018: Journal of Immunological Methods
Shu-Yin Pang, Yu-Mei Dai, Rui-Zhong Zhang, Yi-Hao Chen, Xiao-Fang Peng, Jie Fu, Zheng-Rong Chen, Yun-Feng Liu, Li-Yuan Yang, Zhe Wen, Jia-Kang Yu, Hai-Ying Liu
AIM: To investigate the prevalence and clinical significance of autoimmune liver disease (ALD)-related autoantibodies in patients with biliary atresia (BA). METHODS: Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies (ANAs), by line-blot assay; ANA and anti-neutrophil cytoplasmic antibody (ANCA), by indirect immunofluorescence assay; specific ANCAs and anti-M2-3E, by enzyme linked immunosorbent assay...
January 21, 2018: World Journal of Gastroenterology: WJG
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
Ali Safavi Naini, Jahangir Ghorbani, Sima Montazer Lotfe Elahi, Mohsen Beigomi
Introduction: Granulomatosis with polyangiitis (GPA; also known as Wegener's granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otology manifestations in the disease course. Materials and Methods: In the current prospective study, patients with a diagnosis of GPA from 2012-2016 were included...
November 2017: Iranian Journal of Otorhinolaryngology
Kota Maekawa, Masunari Shibata, Masaru Seguchi, Kazuto Kobayashi, Yutaka Naito, Fumitaka Miya
A 51-year-old man was diagnosed with eosinophilic granulomatosis with polyangiitis 6 years ago due to asthma, sinusitis, hypereosinophilia, and peripheral neuropathy based on the diagnostic criteria of American College of Rheumatology, and corticosteroid therapy achieved a remission. One year ago, he was hospitalized due to deep venous thrombosis (DVT) and pulmonary embolism, and rivaroxaban was administrated. He was admitted to our hospital for acute onset of diplopia and right hemiparesis. Peripheral blood examinations disclosed leukocytosis with hypereosinophilia...
January 17, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
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