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A variety of clinical presentations of eosinophilic granulomatosis with polyangiitis: a comprehensive review.
Reumatologia
December 2024
Department of Rheumatology and Immunology, Jagiellonian University Medical College, Krakow, Poland.
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by eosinophilic granulomatous vasculitis. Typical symptoms include late-onset bronchial asthma and blood and tissue eosinophilia. In addition to these characteristic symptoms, EGPA can affect important organs such as the skin, kidneys, heart, sinuses, gastrointestinal tract, and nervous system.
View Article and Find Full Text PDFMyeloperoxidase anti-neutrophil cytoplasmic antibody-associated vasculitis with silicosis, alveolar hemorrhage, and rapidly progressive glomerulonephritis: a case report.
AME Case Rep
October 2024
Center for Asbestos-Related Diseases, Toyama Rosai Hospital, Toyama, Japan.
Background: The underlying pathophysiology of some occupational diseases such as silicosis involves autoantibodies. An autoantibody, antineutrophil cytoplasmic antibody (ANCA), has been recently reported and is known to be elevated in diseases such as vasculitis; therefore, the disease is currently known as ANCA-associated vasculitis. The risk of ANCA-associated vasculitis is known to be 25 times higher in patients with silicosis than in those without any occupational disease.
View Article and Find Full Text PDFRecommendations 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).
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