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| http://dx.doi.org/10.5021/ad.21.022 | DOI Listing |
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Recommendations for the diagnosis and treatment of anti-neutrophil cytoplasmic autoantibody associated vasculitis.
Nefrologia (Engl Ed)
January 2025
Unidad de Enfermedades Sistémicas Autoinmunes, Departamento de Medicina Interna, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is characterised by small vessel necrotising inflammatory vasculitis. Prior to immunosupressant therapy availability it usually led to a fatal outcome. Current treatment has changed ANCA-associated vasculitis into a condition with a significant response rate, although with a not negligible relapse occurrence and cumulative organ lesions, mostly due to drug-related toxicities.
View Article and Find Full Text PDFRelapsing ANCA-associated vasculitis presenting as a mediastinal mass.
BMJ Case Rep
January 2025
Department of Nephrology, Barts Health NHS Trust, London, UK.
Anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV) represent a heterogeneous multisystem group of disorders typified by necrotising inflammation of smaller blood vessels, classically yielding a pauci-immune, crescentic glomerulonephritis. Without prompt treatment, there is a significant risk of irreversible damage and ensuing renal impairment.Diagnosis is often challenging, exacerbated by the disorder's often vague and insidious presentation.
View Article and Find Full Text PDFConcurrent Takayasu Arteritis and Microscopic Polyangiitis in a Young Female: A Complex Case of Large- and Small-Vessel Vasculitis.
Cureus
December 2024
Internal Medicine, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, Jamaica, USA.
Vasculitides represent a range of disorders marked by inflammation of blood vessels, often posing significant diagnostic challenges due to their diverse clinical presentations and involvement of multiple organ systems. We present the case of a 26-year-old woman who arrived with hemoptysis and a background of exertional dyspnea, chest pain, and occasional visual disturbances. Initial investigations showed elevated perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCAs) and myeloperoxidase antibodies (MPOs), indicative of microscopic polyangiitis (MPA).
View Article and Find Full Text PDFEvaluating Avacopan in the Treatment of ANCA-Associated Vasculitis: Design, Development and Positioning of Therapy.
Drug Des Devel Ther
January 2025
Center of Expertise for Lupus-, Vasculitis- and Complement-Mediated Systemic Diseases (Luvacs), Department of Internal Medicine - Nephrology Section, Leiden University Medical Center, Leiden, the Netherlands.
Recently, avacopan has been approved for the treatment of ANCA-associated vasculitis (AAV). Avacopan is an inhibitor of the C5a-receptor, which plays an important role in chemotaxis and the amplification loop of inflammation in AAV. In the most recent, international guidelines avacopan is recommended as steroid-sparing agents for the management of AAV.
View Article and Find Full Text PDFAdvances in the Assessment and Treatment of Anti-Neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis.
J Inflamm Res
December 2024
Kidney Disease Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, People's Republic of China.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases primarily cause inflammation of small blood vessels. Renal involvement occurs frequently and often leads to end-stage renal disease (ESRD), which significantly impacts patient health and survival. Early diagnosis and appropriate treatment are essential to improving patient outcomes.
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