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Vasculitis ANCA

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https://www.readbyqxmd.com/read/28918161/clinical-and-pathological-significance-of-cutaneous-manifestations-in-anca-associated-vasculitides
#1
REVIEW
Laure Frumholtz, Sara Laurent-Roussel, Olivier Aumaître, François Maurier, Guillaume Le Guenno, Agnes Carlotti, Alexiane Dallot, Jean Louis Kemeny, Laurent Antunes, Nicolas Froment, Sylvie Fraitag, Jonathan London, Alice Berezne, Benoît Terris, Claire Le Jeunne, Luc Mouthon, Selim Aractingi, Loïc Guillevin, Nicolas Dupin, Benjamin Terrier
OBJECTIVES: Cutaneous manifestations (CM) in ANCA-associated vasculitides (AAV) are frequent, but data on clinical significance and clinical-pathological correlations are lacking. METHODS: We conducted a multicenter, retrospective study including 1553 AAV patients. Clinical, biological and pathological features have been analyzed, and tissue samples from 46 biopsies were reviewed in a blind manner. RESULTS: CM were more frequent in EGPA (53...
September 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28917713/the-bvas-is-an-independent-predictor-of-cardiovascular-events-and-cardiovascular-disease-related-mortality-in-patients-with-anca-associated-vasculitis-a-study-of-504-cases-in-a-single-chinese-center
#2
Yi-Hua Bai, Zhi-Ying Li, Dong-Yuan Chang, Min Chen, Cees G M Kallenberg, Ming-Hui Zhao
BACKGROUND: Cardiovascular diseases (CVD) are the major causes of death in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) during long-term follow-up. This study investigated risk factors for cardiovascular events (CVE) and CVD-related mortality in Chinese AAV patients. METHODS: Five hundred and four AAV patients in our center were retrospectively included. The predictive value of variables associated with CVE and CVD-related mortality were analyzed...
August 31, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28916770/changes-in-cd73-cd39-and-cd26-expression-on-t-lymphocytes-of-anca-associated-vasculitis-patients-suggest-impairment-in-adenosine-generation-and-turn-over
#3
Lovis Kling, Urs Benck, Annette Breedijk, Lisa Leikeim, Marianne Heitzmann, Stefan Porubsky, Bernhard K Krämer, Benito A Yard, Anna-Isabelle Kälsch
Extracellular adenosine, generated via the concerted action of CD39 and CD73, contributes to T-cell differentiation and function. Adenosine concentrations are furthermore influenced by adenosine deaminase binding protein CD26. Because aberrant T-cell phenotypes had been reported in anti-neutrophil cytoplasmic auto-antibody (ANCA)-associated vasculitis (AAV) patients, an impaired expression of these molecules on T-cells of AAV patients was hypothesized in the present study. While in AAV patients (n = 29) CD26 was increased on CD4(+) lymphocytes, CD39 and CD73 were generally reduced on patients' T-cells...
September 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28914375/central-nervous-system-involvement-in-patients-with-granulomatosis-with-polyangiitis-a-single-center-retrospective-study
#4
George E Fragoulis, Sophia Lionaki, Aliki Venetsanopoulou, Panayiotis G Vlachoyiannopoulos, Haralampos M Moutsopoulos, Athanasios G Tzioufas
The aims of this study were to estimate the frequency of central nervous system (CNS) involvement in Greek patients with granulomatosis with polyangiitis (GPA) and describe the related clinical characteristics and long-term outcomes of these patients. Medical charts of all ANCA-associated vasculitis patients were retrospectively reviewed, and GPA patients with CNS involvement were identified. Demographics, serological, and clinical features throughout the disease course were recorded. Comparisons of disease characteristics and long-term outcomes were performed between GPA patients with and without CNS involvement...
September 15, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28905856/position-paper-revised-2017-international-consensus-on-testing-of-ancas-in-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#5
REVIEW
Xavier Bossuyt, Jan-Willem Cohen Tervaert, Yoshihiro Arimura, Daniel Blockmans, Luis Felipe Flores-Suárez, Loïc Guillevin, Bernhard Hellmich, David Jayne, J Charles Jennette, Cees G M Kallenberg, Sergey Moiseev, Pavel Novikov, Antonella Radice, Judith Anne Savige, Renato Alberto Sinico, Ulrich Specks, Pieter van Paassen, Ming-Hui Zhao, Niels Rasmussen, Jan Damoiseaux, Elena Csernok
Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications...
September 14, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28899799/neutrophil-extracellular-traps-nets-in-autoimmune-diseases-a-comprehensive-review
#6
REVIEW
Keum Hwa Lee, Andreas Kronbichler, David Duck-Young Park, YoungMin Park, Hanwool Moon, Hyungdo Kim, Jun Hyug Choi, YoungSeo Choi, Songjoo Shim, Il Suk Lyu, Byung Hwan Yun, Yeonseung Han, Donghee Lee, Sang Yoon Lee, Byung Hun Yoo, Kyung Hwan Lee, Tai Lim Kim, Heonki Kim, Joo Sung Shim, Wonseok Nam, Heesung So, SooYeon Choi, Sangmok Lee, Jae Il Shin
The structures named neutrophil extracellular traps (NETs) are fibrous networks which protrude from the membrane of activated neutrophils. NETs are found in a variety of conditions, such as infection, malignancy, atherosclerosis, and autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), psoriasis, and gout. The impact of NETs on the development mechanisms of autoimmune diseases are proposed to arise from an imbalance between "NETosis" which is a process of NET formation and NET degradation...
September 9, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28895041/unmet-needs-in-the-pathogenesis-and-treatment-of-vasculitides
#7
REVIEW
Francesco Muratore, Giulia Pazzola, Alessandra Soriano, Nicolò Pipitone, Stefania Croci, Martina Bonacini, Luigi Boiardi, Carlo Salvarani
Despite the progress in the last years on the field of vasculitides, there are several unmet needs regarding classification, disease activity assessment, predictors of flares and complications, and type of treatment for the different forms. The 1990 American College of Rheumatology (ACR) classification criteria currently used to define giant cell arteritis and Takayasu arteritis were designed to discriminate between different types of vasculitides but not to differentiate vasculitis from other disorders. Recently, efforts have been made to overcome the shortcomings of the ACR criteria...
September 11, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28885482/comparison-of-localized-and-systemic-otitis-media-with-anca-associated-vasculitis
#8
Masahiro Okada, Koichiro Suemori, Daiki Takagi, Masato Teraoka, Hiroyuki Yamada, Naohito Hato
OBJECTIVE: To investigate differences in immune activity based on the presence of multiple organ involvement in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and whether hearing outcomes are different between patients with AAV localized to the ear and patients with systemic AAV. STUDY DESIGN: Retrospective case review. SETTING: University hospital. PATIENTS: Twenty patients with otitis media with AAV (OMAAV) who met the criteria proposed by the OMAAV study group in Japan...
September 6, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28884919/granulomatosis-with-polyangiitis-presenting-with-pyoderma-gangrenosum-like-ulceration-and-negative-cytoplasmic-antineutrophilic-cytoplasmic-antibodies-in-a-child
#9
Ashley Kass, Jake D Fagan, Paul Long
Granulomatosis with polyangiitis (GPA), a vasculitis that most commonly affects small to medium-size vessels of the respiratory tract and kidneys, may also present with a wide array of skin findings. We present the case of a 12-year-old boy with pyoderma gangrenosum-like ulcerations on his lower extremity as the initial manifestation of GPA despite negative cytoplasmic antineutrophilic cytoplasmic antibodies (c-ANCAs). Although GPA is strongly associated with c-ANCA, c-ANCA may be negative on presentation. Thus clinical and pathologic clues must be relied upon when serologic confirmation is negative...
September 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28883245/the-sequential-development-of-antiglomerular-basement-membrane-nephritis-and-myeloperoxidase-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#10
Naro Ohashi, Akio Namikawa, Masafumi Ono, Takamasa Iwakura, Shinsuke Isobe, Takayuki Tsuji, Akihiko Kato, Hideo Yasuda
A 55-year-old woman presented with deafness, increased levels of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA), and renal insufficiency with proteinuria and hematuria. Renal biopsy revealed crescentic glomerulonephritis with the linear deposition of immunoglobulin G along the glomerular basement membrane (GBM) and peritubular capillaritis. The anti-GBM antibody levels on admission and 10 days after admission were 11.7 U/mL and 127 U/mL, respectively. These results indicated the sequential development of anti-GBM nephritis and MPO-ANCA-associated vasculitis...
September 6, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28881714/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-years-observation-study
#11
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
August 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28881446/the-epidemiology-of-anca-associated-vasculitis-in-olmsted-county-minnesota-usa-a-20-year-population-based-study
#12
Alvise Berti, Divi Cornec, Cynthia S Crowson, Ulrich Specks, Eric L Matteson
OBJECTIVE: To estimate the incidence, prevalence and mortality of ANCA-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), in a U.S. based adult population. METHODS: All medical records of patients with a diagnosis or suspicion of AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. Incidence rates were age- and sex-adjusted to the 2010 US white population...
September 7, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28881339/interstitial-immunostaining-and-renal-outcomes-in-antineutrophil-cytoplasmic-antibody-associated-glomerulonephritis
#13
Duvuru Geetha, Sanjeev Sethi, An S De Vriese, Ulrich Specks, Cees G M Kallenberg, Noha Lim, Robert Spiera, E William St Clair, Peter A Merkel, Philip Seo, Paul A Monach, Nicola Lepori, Barri J Fessler, Carol A Langford, Gary S Hoffman, Rishi Sharma, John H Stone, Fernando C Fervenza
BACKGROUND: Immunopathologic features predict renal function at baseline and follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN). The interstitial infiltrate consists predominantly of T lymphocytes, but their pathophysiologic significance is unclear, especially in light of the success of B-cell-directed therapy. METHODS: Renal biopsies from 33 patients treated with cyclophosphamide (CYC; n = 17) or rituximab (RTX; n = 16) in the RTX in ANCA-associated vasculitis (RAVE) trial were classified according to the new ANCA GN classification...
September 8, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28880680/the-effect-and-possible-clinical-efficacy-of-in-vivo-inhibition-of-neutrophil-extracellular-traps-by-blockade-of-pi3k-gamma-on-the-pathogenesis-of-microscopic-polyangiitis
#14
Hirotaka Kimura, Yasushi Matsuyama, Sachiko Araki, Atsushi Koizumi, Yumi Kariya, Shunsuke Takasuga, Satoshi Eguchi, Hiroki Nakanishi, Junko Sasaki, Takehiko Sasaki
OBJECTIVE: Neutrophil extracellular traps (NETs) are peculiar structures composed of the externalized chromatin with intracellular proteins and formed by activated neutrophils in a reactive oxygen species (ROS)-dependent manner. Aberrant NETs are considered to be autoantigens for anti-neutrophil cytoplasmic antibodies (ANCAs) underling the development of microscopic polyangiitis (MPA). However, little is known regarding the therapeutic efficacy of in vivo inhibition of NET formation (NETosis) on MPA pathogenesis...
September 7, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28878204/analysis-of-antineutrophil-cytoplasm-antibody-from-118-730-patients-in-tertiary-hospitals-in-jiangxi-province-china
#15
Liming Tan, Anjun Jiao, Juanjuan Chen, Xiaojing Feng, Liuyue Xu, Siqi He, Fuyan Tan, Yongqing Jiang, Heng Luo, Hua Li, Yang Wu, Yongjian Tian, Tingting Zeng, Jianlin Yu, Liping Cao, Jianfeng Zheng, Hui Xu, Ming Wei, Wen Gan, Weihua Peng, Yanming Liu, Jing Hou, Jiangxia Xu, LiHua Shuai, Wenzhi Huang, Junyun Huang, Yan Lin, Jianrong Liu
BACKGROUND The discovery of antineutrophil cytoplasm antibody (ANCA) makes the early diagnosis of primary vasculitis possible, and also has important guiding significance for the diagnosis and treatment of secondary vasculitis. This study aimed to investigate the clinical significance of ANCA. MATERIAL AND METHODS ANCA was detected by indirect immunofluorescence assay (IIF), and anti-myeloperoxidase (MPO) antibody, and anti-proteinase 3 (PR3) antibody were detected by ELISA. The results were analyzed retrospectively...
September 7, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28870527/identification-and-phenotyping-of-circulating-autoreactive-proteinase-3-specific-b-cells-in-patients-with-pr3-anca-associated-vasculitis-and-healthy-controls
#16
Divi Cornec, Alvise Berti, Amber Hummel, Tobias Peikert, Jacques-Olivier Pers, Ulrich Specks
OBJECTIVES: To develop a method to detect and phenotype circulating proteinase 3 (PR3)-specific B-cells in patients with PR3-ANCA associated vasculitis (AAV). METHODS: Recombinant human PR3 (rPR3) was tagged with FITC or biotin, and its binding characteristics were studied by flow cytometry using three hybridoma cell lines secreting antibodies (Ab) against human PR3, mouse PR3 (no cross-reactivity with human PR3), and human neutrophil elastase. We measured the proportion of PR3-specific B-cells and studied their surface phenotype in patients with PR3-AAV and healthy controls (HC)...
September 1, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28868585/spectrum-of-anca-associated-vasculitis
#17
REVIEW
Aditi Sinha, Arvind Bagga
No abstract text is available yet for this article.
September 4, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28868297/a-toddler-presenting-with-pulmonary-renal-syndrome
#18
Florence A Aeschlimann, Rae S M Yeung, Ronald M Laxer, Diane Hebert, Ashley Cooper, Rose Chami, Damien Noone
Pulmonary renal syndrome refers to an association of pulmonary and glomerular disease and includes disorders, such as the ANCA-associated vasculitides, anti-glomerular basement membrane antibody disease, systemic lupus erythematosus, and IgA vasculitis (Henoch-Schönlein purpura). We present the medical history of a 26-month-old boy with an extensive purpuric rash, involving the limbs, trunk, and face, who developed clinically significant pulmonary hemorrhage and renal involvement. Rapid recognition of this rare but potentially life-threatening condition is crucial...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28866833/voriconazole-induced-periostitis-deformans-serial-imaging-in-a-patient-with-anca-vasculitis
#19
S Cormican, N Adams, P O'Connell, A McErlean, D de Freitas
OBJECTIVES: A 61-year-old with acute granulomatosis and polyangiitis developed Aspergillus fumigatus pneumonia after admission to the intensive care unit with a small bowel perforation. This occurred after immunosuppression (intravenous methylprednisolone, intravenous cyclophosphamide, and plasmapheresis) for his initial presentation with stage 3 acute kidney injury. MATERIALS AND METHODS: The mycologist recommended long-term treatment with voriconazole after initial recovery...
September 2, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28864843/pupillary-autonomic-dysfunction-in-patients-with-anca-associated-vasculitis
#20
Philipp Moog, O Eren, S Kossegg, K Valda, A Straube, M Grünke, H Schulze-Koops, M Witt
OBJECTIVE: To assess autonomic function by infrared dynamic pupillometry in patients with ANCA-vasculitis (AAV) in correlation to autonomic symptoms, disease specific clinical parameters and cardiovascular reflex tests. METHODS: Patients with AAV and healthy controls underwent pupillometry at rest and after sympathetic stimulation (cold pressor test). Three parasympathetic parameters (amplitude, relative amplitude, maximum constriction velocity) and one sympathetic parameter (late dilatation velocity) were assessed...
September 1, 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
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