Mixed Cryoglobulinemic Vasculitis: A Case Report.
Mixed Cryoglobulinemic Vasculitis: A Case Report. J Nippon Med Sch. 2018;85(2):68-69 Authors: Hirama A, Mii A, Arakawa Y, Funakoshi T, Ko M, Sakai Y, Shimizu A, Tsuruoka S PMID: 29731499 [PubMed - in process]
AbstractBackgroundIn antineutrophil cytoplasmic antibody (ANCA) –associated vasculitis (AAV), central nervous system (CNS) involvement is relatively uncommon. The current study retrospectively investigated the clinical features and outcomes of AAV patients with CNS involvement.MethodsA total of 497 AAV patients were retrospectively recruited in our center, twenty-nine of which had CNS involvement. Clinical and radiological manifestations and the outcomes of these patients were analyzed.ResultsThe predominant symptom was sensorimotor impairment. According to the MRI findings, twenty-four patients had cerebral ischemic...
ConclusionIn this large international study, several clinical and laboratory factors were identified as associated with renal involvement in ANCA-associated vasculitis.
Background: Although an etiology of Kawasaki disease (KD) is unknown, an aberrant innate immune system in predisposed individuals has been proposed to play a key role in the development of KD vasculitis. Various etiological pathogens have been propo...
Publication date: Available online 13 February 2020Source: CellAuthor(s): Mandeep Singh, Katherine J.L. Jackson, Jing J. Wang, Peter Schofield, Matt A. Field, David Koppstein, Timothy J. Peters, Deborah L. Burnett, Simone Rizzetto, Damien Nevoltris, Etienne Masle-Farquhar, Megan L. Faulks, Amanda Russell, Divya Gokal, Asami Hanioka, Keisuke Horikawa, Alexander D. Colella, Timothy K. Chataway, James Blackburn, Tim R. MercerSummaryPathogenic autoantibodies arise in many autoimmune diseases, but it is not understood how the cells making them evade immune checkpoints. Here, single-cell multi-omics analysis demonstrates a share...
Abstract Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) form a group of necrotizing small-vessel vasculitides characterized by the presence of ANCA against either proteinase 3 (PR3) or myeloperoxidase (MPO). ANCA have a key role in the pathogenesis of AAV, inducing excessive activation of neutrophils, which results in injury to small vessels1 ANCA can target other neutrophil-derived molecules, among them lysosome-associated membrane glycoprotein 2 (LAMP-2). PMID: 32062605 [PubMed - as supplied by publisher]
This study identifies clinical features that should alert physicians to the possibility of an underlying metabolic disease. Treatment of the metabolic disease leads to symptomatic improvement.
In findings that raise questions about the go-to treatment for patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, researchers behind the PEXIVAS study have concluded that plasma exchange does not reduce the odds of death or end-stage kidney disease.Reuters Health Information
ConclusionRTX seems to be a safe and useful agent in inducing disease remission and reducing previous immunosuppressive treatment in IgAV pediatric and adult patients resistant or refractory to glucocorticoids or other immunosuppressive drugs, and in those patients in whom these agents are contraindicated. Nevertheless, controlled clinical trials in are still warranted to clarify the role of RTX in IgAV.
ConclusionsThe majority of patients with PCNSV responded to treatment. We found patient subsets with different outcomes. Mycophenolate mofetil may be an effective alternative to CYC.
"Fleshy" Skin Cellulitis: A Triggering Factor for ANCA Associated Vasculitis. Acta Med Indones. 2019 Oct;51(4):338-343 Authors: Yang SC, Mustafar R, Kamaruzaman L, Wei Yen K, Mohd R, Cader R Abstract A 59-year-old lady with underlying hypothyroidism presented with acute contact dermatitis progressed to cellulitis with superimposed bacterial infection and acute kidney injury. She responded to initial management with antibiotics, but a week later, she had cutaneous and systemic vasculitis. Her skin biopsy consistent with immune-mediated leuko-cytoclastic vasculitis and her blood test was positi...
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