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Relapsing 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 PDFBackground: Right ventricular pseudoaneurysms are extremely rare and there is no precise recommendation for their management.
Case Summary: We present the case of a successful coil embolization of a right ventricular pseudoaneurysm in a woman with chronic kidney disease due to perinuclear antineutrophil cytoplasmic antibody vasculitis and a history of difficult placement of a hemodialysis catheter.
Discussion: Ventricular pseudoaneurysm is a contained rupture of the ventricular wall that can occur due to several causes.
Concurrent 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 PDFA Rare Case of Spinal Hypertrophic Pachymeningitis That Caused Tetraplegia After Myelography.
Cureus
December 2024
Department of Orthopaedics, Tokyo Metropolitan Bokutoh Hospital, Tokyo, JPN.
Hypertrophic pachymeningitis (HP) is a rare inflammatory disease that causes the thickening of the dura mater. Its etiology is mainly classified as idiopathic or secondary, and autoimmune disease is one of the main causes of secondary HP. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4-related disease are common among autoimmune diseases.
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.
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