Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare multisystem autoimmune condition that causes inflammation of small and medium-sized blood vessels and is more commonly seen in the geriatric population. ANCA-associated vasculitis (AAV) is typically characterized as necrotizing vasculitis and includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). The mortality rate remains high, with especially cardiovascular disease, infections, and malignancies being the leading causes of death. Existing treatment options depend heavily on the use of glucocorticoids (GCs), often in combination with cyclophosphamide (CYC); however, as the multitude of adverse effects associated with these agents has increased, numerous studies are being conducted to reduce not only these harmful effects but also improve remission rates. Rituximab, avacopan, corticosteroids, including intravenous pulse methylprednisolone, plasma exchange, and immunological targeting are among the emerging treatments. The purpose of this review is to emphasize the pathogenesis and traditional treatment modalities and give insights into the recent advances in managing this disorder in an attempt to spare the adverse effects of conventional therapies while achieving better remission rates with combination therapies as well. The authors explored multiple databases, employing appropriate keywords, satisfying the quality appraisal, after which a total of 14 reports were included in this review. Upon overall analysis, it can be concluded that rituximab and CYC, when used in combination, provided a safer alternative to GCs while exhibiting equal, if not superior, effectiveness and results, thus, paving the way for additional in-depth research in a larger population of interest.
Highlights
BackgroundVasculitis is a disorder in which the blood vessels become inflamed [1], resulting in vessel wall thickening and a reduction in the amount of blood that can flow through them [2]
Leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO) are the two primary antigens targeted by Anti-neutrophil cytoplasmic antibody (ANCA) [3], which are found on the membranes of activated neutrophils and monocytes [4]
A literature search was done on the PubMed, Google Scholar, Science Direct, and Cochrane Library databases using regular and medical subject heading (MeSH) keywords through the Boolean scheme, as listed below
Summary
BackgroundVasculitis is a disorder in which the blood vessels become inflamed [1], resulting in vessel wall thickening and a reduction in the amount of blood that can flow through them [2]. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe and chronic condition that affects the small blood vessels in the body and is characterized by autoantibodies that target neutrophils. The etiology of ANCA-associated vasculitis (AAV) has remained multifactorial and is thought to be affected by factors such as genetics, environmental conditions [1], infections [4], and innate/acquired immunity [1]. Recent advancements in the treatment and management of AAV have dramatically increased patient prognosis in recent years [3], including the use of rituximab, avacopan, plasma exchange, immunological targeting, and intravenous pulse methylprednisolone, with all demonstrating success in achieving remission [2,4,5,6,7,8,9]. The purpose of this review is to provide relevant background data regarding the prevalence, significance, pathophysiology of AAV, and traditional treatment modalities while describing the contemporary advancements in this disorder's treatment and management capabilities
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