This article focuses on the initial results achieved with the more selective immunosuppressive approach of B-lymphocyte depletion in patients who fail cyclophosphamide or have contraindications for its use in the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This novel approach has sparked hope for patients and physicians in their search for effective, well-tolerated therapy for AAV. B-cell depletion is now undergoing rigorous investigation in randomized clinical trials.
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Reclassification of the overlap syndrome of Behçet's disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet's disease.
Korean J Intern Med
January 2025
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Background/aims: This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with Behçet's disease (BD) to investigate the proportion and clinical implications of the reclassification to the overlap syndrome of BD and AAV (OS-BD-AAV).
Methods: We included 280 BD patients presenting with ANCA positivity but without medical conditions mimicking AAV at diagnosis. Demographic data, items from the 2014 revised International Criteria for BD and 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology criteria for AAV, ANCA positivity, and laboratory results were recorded as clinical data at diagnosis.
Childhood-Onset ANCA-Associated Vasculitis: From Genetic Studies to Advances in Pathogenesis, Classification and Novel Therapeutic Approaches.
Int J Mol Sci
December 2024
Department of Adolescent and Young Adult Rheumatology, University College London NHS Foundation Trust, London NW1 2PG, UK.
Childhood-onset antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) represents a heterogeneous group of multi-system autoimmune conditions associated with chronic inflammation, characteristically affecting small blood vessels, leading to various organ and system manifestations. Although rare in paediatric populations, AAV poses challenges in early recognition, diagnosis and management of refractory cases. This review highlights the characteristics of clinical presentation and outcomes of AAV in children, as well as its current classification and progress achieved in understanding the disease pathogenesis, with a focus on adult and paediatric genetic studies.
View Article and Find Full Text PDFComparison of the clinicopathological characteristics of children with anti-neutrophil cytoplasmic antibody-associated vasculitis with/without infection at diagnosis.
BMC Nephrol
January 2025
Department of Nephrology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, 510623, China.
Background: Infectious episodes contribute to morbidity and mortality in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Renal involvement, also known as ANCA-associated glomerulonephritis (AGN), is frequently observed in AAV. Little is known about whether co-infection at initial diagnosis is associated with renal outcome and prognosis in children with AGN.
View Article and Find Full Text PDFBurden of coronary artery calcification in ANCA-associated vasculitis.
RMD Open
January 2025
Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA
Background: Cardiovascular disease (CVD) is a leading cause of death in ANCA-associated vasculitis (AAV). Screening and primary cardiovascular prevention may improve outcomes.
Methods: We identified patients in the 2002-2019 Mass General Brigham AAV cohort with thoracic CT scans obtained for other clinical purposes.
Granulomatosis With Polyangiitis in a Young Lady: Challenges in Achieving Remission.
Cureus
December 2024
Ophthalmology, Universiti Kebangsaan Malaysia Medical Centre (UKMMC), Kuala Lumpur, MYS.
Granulomatosis with polyangiitis (GPA) is a subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that commonly requires aggressive immunosuppression to achieve remission. We present a case of a young Malay lady with recurrent episodes of ANCA-positive nodular anterior scleritis who responded poorly to topical and systemic corticosteroids and relapsed while on methotrexate. A year later, she had epistaxis, and a sino-nasal biopsy confirmed granulomatous vasculitis.
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