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ANCA vasculitis

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https://www.readbyqxmd.com/read/29147477/vanishing-vasculitis-a-case-of-acute-necrotic-skin-findings-without-pathologic-features-of-vasculitis-from-adulterated-cocaine
#1
Adnan Asif Parvez Ghias, Patrick Brine
While the usage of illicit drugs in itself carries significant health risks and associated toxicities, drugs that are adulterated to give them volume, alter their psychogenic properties, and make them cheaper to produce are to be considered even more dangerous. Cocaine is one of them, and it is now most commonly being adulterated with levamisole. We report a case of a 37-year-old female with the chief complaint of painful skin lesions and wounds on both of her upper and lower extremities for three weeks duration...
2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29141676/platelets-are-activated-in-anca-associated-vasculitis-via-thrombin-pars-pathway-and-can-activate-the-alternative-complement-pathway
#2
Di Miao, Dan-Yang Li, Min Chen, Ming-Hui Zhao
BACKGROUND: In this study, we investigated the mechanism of platelet activation in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), as well as the activation of the alternative complement pathway by platelets in AAV. METHODS: CD62P and platelet-leukocyte aggregates in AAV patients were tested by flow cytometry. Platelets were stimulated by plasma from active AAV patients. The effect of the thrombin-protease-activated receptors (PARs) pathway was evaluated by blocking thrombin or PAR1 antagonists...
November 15, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#3
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29136340/dermatological-addendum-to-the-2012-international-chapel-hill-consensus-conference-nomenclature-of-vasculitides
#4
Cord H Sunderkötter, Bernhard Zelger, Ko-Ron Chen, Luis Requena, Warren Piette, J Andrew Carlson, Jan Dutz, Peter Lamprecht, Alfred Mahr, Elisabeth Aberer, Victoria P Werth, David A Wetter, Seiji Kawana, Raashid Luqmani, Camille Frances, Joseph Jorizzo, J Richard Watts, Dieter Metze, Marzia Caproni, Erkan Alpsoy, Jeffrey P Callen, David Fiorentino, Peter A Merkel, Ronald J Falk, J Charles Jennette
OBJECTIVE: The objective of this project was to prepare a dermatologic addendum to the 2012 Chapel Hill consensus conference nomenclature to address vasculitides affecting the skin. The goal was to standardize names and definitions for cutaneous vasculitis. METHODS: A nominal group technique with a facilitator was used to reach a consensus using multiple face-to-face meetings, e-mail discussions, and teleconferences. RESULTS: Standardized names, definitions and descriptions are provided for cutaneous components of systemic vasculitides (e...
November 14, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29124398/patient-perceptions-of-glucocorticoids-in-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#5
Joanna C Robson, Jill Dawson, Peter F Cronholm, Susan Ashdown, Ebony Easley, Katherine S Kellom, Don Gebhart, Georgia Lanier, Nataliya Milman, Jacqueline Peck, Raashid A Luqmani, Judy A Shea, Gunnar Tomasson, Peter A Merkel
Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are multisystem diseases of small blood vessels, collectively known as the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). This study explores the patient's perspective on the use of glucocorticoids, which are still a mainstay of treatment in AAV. Patients with AAV from the UK, USA, and Canada were interviewed, using purposive sampling to include a range of disease manifestations and demographics...
November 9, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29119480/rheumatoid-factor-false-positivity-in-patients-with-anca-associated-vasculitis-not-having-medical-conditions-producing-rheumatoid-factor
#6
Jae-Seung Moon, Diane Da-Hyun Lee, Yong-Beom Park, Sang-Won Lee
We investigated the rate of rheumatoid factor (RF) false positivity at diagnosis and its influence on clinical and prognostic features and rheumatoid arthritis (RA) development during the follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients without RA or other medical conditions triggering RF false positivity. We reviewed the medical records of 128 AAV patients. We divided patients with AAV and each variant into two groups according to RF positivity and compared variables between the two groups...
November 8, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29108823/cocaine-and-anca-associated-vasculitis-like-syndromes-a-case-series
#7
REVIEW
Sujith Subesinghe, Sander van Leuven, Leena Yalakki, Shirish Sangle, David D'Cruz
OBJECTIVES: We analysed the spectrum of clinical manifestations of cocaine associated pseudovasculitis. METHODS: Clinical, serological, radiological and histological features of 14 patients with cocaine pseudovasculitis syndromes were included. RESULTS: Twelve patients had significant sinus thickening or erosive disease. Other multi-system manifestations included vasculitic rashes, pulmonary lesions and peripheral neuropathy. All patients had positive ANCA titres at presentation...
November 3, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29106654/the-population-level-costs-of-immunosuppression-medications-for-the-treatment-of-glomerulonephritis-are-increasing-over-time-due-to-changing-patterns-of-practice
#8
Sean Barbour, Clifford Lo, Gabriela Espino-Hernandez, Sharareh Sajjadi, John Feehally, Scott Klarenbach, Jagbir Gill
Background: Immunosuppression (IS) is the main treatment for most types of glomerulonephritis (GN). Quantifying the cost of IS is necessary to ensure equitable access to therapies and optimal health outcomes, but the real-world cost of IS treatment for GN is largely unknown. We examined temporal changes in the population-level IS medication costs for GN over a 14-year period in a large Canadian province. Methods: We linked a provincial pathology database (containing all GN cases from 2000 to 2012) with renal and medication administrative databases to capture clinical characteristics and IS medications, with follow-up until 2013...
July 2, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29104431/a-case-of-rare-diffuse-alveolar-hemorrhage-and-review-of-literature
#9
Viviana Scollo, Luca Zanoli, Elisa Russo, Giulio Distefano, Francesco Rapisarda
It is well known that some disorders can cause concomitant kidney dysfunction with lung involvement. These syndromes, characterized by the simultaneous presence of intra-alveolar hemorrhage and acute glomerulonephritis, are caused by numerous and variable disorders. The most frequent are the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and Goodpasture syndrome. A quick discerning of the underlying causes and initiation of adequate treatment is crucial to prevent acute respiratory failure and irreversible loss of renal function...
2017: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29100671/-nettling-the-host-breaking-of-tolerance-in-chronic-inflammation-and-chronic-infection
#10
REVIEW
Sladjana Skopelja-Gardner, Jonathan D Jones, William F C Rigby
How and why we break tolerance to self-proteins still remains a largely unanswered question. Neutrophils have been identified as a rich source of autoantigens in a wide array of autoimmune diseases that arise as a consequence of different environmental and genetic factors, e.g. rheumatoid arthritis (RA), lupus, vasculitis, cystic fibrosis (CF) etc. Specifically, neutrophil extracellular trap (NET) formation has been identified as a link between innate and adaptive immune responses in autoimmunity. Autoantigens including neutrophil granular proteins (targeted by anti-neutrophil cytoplasmic antibodies, ANCA) as well as post-translationally modified proteins, i...
October 31, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29093401/diffuse-alveolar-hemorrhage-developing-immediately-after-immunosuppressive-treatments-in-a-patient-with-granulomatosis-with-polyangiitis-who-had-pulmonary-nodules
#11
Chiaki Watanabe, Shoichi Fukui, Naoki Iwamoto, Toshimasa Shimizu, Masataka Umeda, Ayako Nishino, Tomohiro Koga, Shin-Ya Kawashiri, Kunihiro Ichinose, Yasuko Hirai, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Kazuhiro Tabata, Atsushi Kawakami
A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the detection of high MPO-ANCA, vasculitis and granulomas in a lung biopsy specimen and crescentic glomerulonephritis in a kidney biopsy specimen. Soon after the initiation of intravenous methylprednisolone pulse therapy (mPSL pulse) and intravenous cyclophosphamide pulse therapy (IVCY), the patient experienced cough and hemoptysis. Based on emerging anemia and bilateral diffuse lung consolidation on computed tomography, we judged that diffuse alveolar hemorrhage (DAH) was complicated by GPA...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29088997/clinical-characteristics-and-outcomes-of-propylthiouracil-induced-antineutrophil-cytoplasmic-antibody-associated-vasculitis-in-patients-with-graves-disease-a-median-38-month-retrospective-cohort-study-from-a-single-institution-in-china
#12
Jun Yang, Lin-Peng Yao, Mengjie Dong, Qin Xu, Jun Zhang, Wan-Wen Weng, Feng Chen
BACKGROUND: This study sought to investigate the clinical characteristics and outcomes of propylthiouracil (PTU)-induced antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in patients with Graves' disease. METHODS: Sixteen patients diagnosed with PTU-induced ANCA-associated vasculitis in our hospital from January 2010 to June 2017 were analyzed retrospectively. RESULTS: All sixteen patients with PTU-induced ANCA-associated vasculitis were female...
October 31, 2017: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/29082297/distinct-pathogenesis-in-nonsystemic-vasculitic-neuropathy-and-microscopic-polyangiitis
#13
Mie Takahashi, Haruki Koike, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Atsushi Hashizume, Masahisa Katsuno, Gen Sobue
OBJECTIVE: To investigate the mechanisms of vasculitis in nonsystemic vasculitic neuropathy (NSVN) and microscopic polyangiitis (MPA), focusing on complement- and antineutrophil cytoplasmic antibody (ANCA)-associated pathogenesis. METHODS: Sural nerve biopsy specimens taken from twenty-four patients with NSVN and 37 with MPA-associated neuropathy (MPAN) were examined. Twenty-two patients in the MPAN group tested positive for ANCA. RESULTS: Immunostaining for complement component C3d deposition showed more frequent positive staining of epineurial small vessels in NSVN than in MPAN (p = 0...
November 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29078325/necroptosis-controls-net-generation-and-mediates-complement-activation-endothelial-damage-and-autoimmune-vasculitis
#14
Adrian Schreiber, Anthony Rousselle, Jan Ulrich Becker, Anne von Mässenhausen, Andreas Linkermann, Ralph Kettritz
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes life-threatening autoimmune diseases affecting every organ, including the kidneys, where they cause necrotizing crescentic glomerulonephritis. ANCA activates neutrophils and activated neutrophils damage the endothelium, leading to vascular inflammation and necrosis. Better understanding of neutrophil-mediated AAV disease mechanisms may reveal novel treatment strategies. Here we report that ANCA induces neutrophil extracellular traps (NETs) via receptor-interacting protein kinase (RIPK) 1/3- and mixed-lineage kinase domain-like (MLKL)-dependent necroptosis...
October 24, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29071483/-s1-guidelines-on-diagnostics-and-treatment-of-anca-associated-vasculitis
#15
EDITORIAL
Jan H Schirmer, Frank Moosig
No abstract text is available yet for this article.
October 25, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/29061248/involvement-of-the-peripheral-nervous-system-in-polyarteritis-nodosa-and-antineutrophil-cytoplasmic-antibodies-associated-vasculitis
#16
REVIEW
John B Imboden
Peripheral nerve involvement is common in polyarteritis nodosa and the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. The underlying mechanism is arteritis of the vasa nervorum, leading to ischemic neuropathy. The classic presentation is stepwise involvement of peripheral nerves with ongoing antecedent constitutional symptoms. This article reviews the pathologic findings, clinical syndromes, diagnosis, and treatment of ANCA-associated vasculitides.
November 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29056777/relapsing-granulomatosis-with-polyangiitis-with-severe-lung-and-upper-respiratory-tract-involvement-successfully-treated-with-rituximab
#17
Anna Masiak, Zbigniew Zdrojewski
Granulomatosis with polyangiitis (GPA) is a chronic, relapsing, primary systemic small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies. The disease is characterised by the involvement of various organs. The relapse rate rises from about 20% at 12 months to about 60% at 5 years. The combination of glucocorticoids and cyclophosphamide remains the standard therapy for patients with generalised GPA; nevertheless, some patients do not respond satisfactorily to this treatment. According to EULAR-EDTA recommendations for the management of ANCA-associated vasculitis, RTX should be considered for remission-induction of new-onset as well as major relapse of organ-threatening or life-threatening GPA...
2017: Reumatologia
https://www.readbyqxmd.com/read/29045980/-anti-neutrophil-cytoplasmic-antibodies-associated-vasculitis-with-lung-hemorrhage-in-the-patient-on-maintenance-haemodialysis-a-case-report
#18
J X Ding, M Wang
Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is usually a multisystem disorder, and pulmonary renal syndrome is a common presentation. Patients with AAV are less likely to experience relapse when they progress to end-stage renal disease (ESRD). We report a rare case of diffuse alveolar hemorrhage (DAH) in relapsing AAV after eight years of haemodialysis. A 58-year-old woman was admitted to our hospital with the chief complaints of dyspnea and hemoptysis accompanied by anemia, fever, fatigue, and weight loss...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29045715/towards-precision-medicine-in-anca-associated-vasculitis
#19
Kornelis S M van der Geest, Elisabeth Brouwer, Jan-Stephan Sanders, Maria Sandovici, Nicolaas A Bos, Annemieke M H Boots, Wayel H Abdulahad, Coen A Stegeman, Cees G M Kallenberg, Peter Heeringa, Abraham Rutgers
ANCA-associated vasculitis (AAV) is characterized by inflammation and destruction of small and medium-sized vessels. Current management strategies for AAV have been validated in large groups of patients. However, recent insights indicate that distinct patient subsets may actually exist within AAV, thereby justifying the development of more personalized treatment strategies. In this review, we discuss current evidence for a better classification of AAV based on ANCA type. We describe how thus defined categories of AAV patients may differ in genetic background, clinical presentation, immune pathology, response to treatment and disease outcome...
October 17, 2017: Rheumatology
https://www.readbyqxmd.com/read/29043145/anca-associated-pauci-immune-glomerulonephritis-in-a-patient-with-bacterial-endocarditis-a-challenging-clinical-dilemma
#20
Andrea Cervi, Dylan Kelly, Iakovina Alexopoulou, Nader Khalidi
PURPOSE: We report the case of a 59-year-old man with chronic hepatitis B and C infection presenting with acute kidney injury and enterococcus faecalis-infective endocarditis (IE). An elevated proteinase-3 (PR3)-ANCA and pauci-immune glomerulonephritis (GN) on renal biopsy were discovered, corresponding to ANCA-mediated GN. We conducted a literature review to assess the role of ANCA in IE and treatment implications. METHODS: On systematic review of the literature, we found five previous cases whereby IE caused by streptococcus and bartonella species were related to ANCA vasculitis-associated GN...
2017: Clin Nephrol Case Stud
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