Recent Advancements in the Management of Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review

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.

Two Rare Diseases, One Patient: A Case Report of Mucormycosis and Granulomatous Polyangiitis

“…GPA is a rare aseptic necrotizing granulomatous vasculitis of small and medium-sized blood vessels belonging to a group of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) [ 2 , 3 ]. Cytoplasmic-ANCA (c-ANCA) is the most specific marker for GPA and is seen in 80–90% of GPA cases [ 2 ].…”

“…Cytoplasmic-ANCA (c-ANCA) is the most specific marker for GPA and is seen in 80–90% of GPA cases [ 2 ]. Combination therapies using glucocorticoids and CYC [ 2 , 3 ], or rituximab and CYC [ 3 ] have shown to be successful. Moreover, plasmapheresis is indicated in patients with severe disease that is unresponsive to intravenous glucocorticoids [ 2 ].…”

“…It is a type of antineutrophil-cytoplasmic-antibody (ANCA)-associated vasculitis (AAV). The global incidence of GPA is approximately 10–20 cases per one million annually [ 2 ] with no gender predisposition but mainly occurs in the older population [ 2 , 3 ]. Diagnosis requires thorough and focused history, a clinical exam, an autoimmune profile, and radiological and histopathological analyses [ 2 ].…”

Vasculitis asociadas a ANCA: análisis de 26 pacientes

“…Entre ellos, algunos prometedores son los niveles séricos de proteosoma 20S, el CD163 soluble en orina, el suPAR, pentraxina-3 y algunos más tradicionales que están mostrando alguna utilidad, como el índice de masa corporal y el ratio eritrosedimentación/PCR, entre otros [55][56][57][58][59][60][61][62][63] . Desde la introducción de la ciclofosfamida y las altas dosis de corticoides, las vasculitis asociadas a ANCA se han transformado de una enfermedad que de 90% de mortalidad al año pasó a ser una enfermedad crónica con remisiones y recaídas 29,[64][65][66] . Actualmente la mortalidad a 5 años es del 25% y la tasa de recaída del 50%.…”

“…El uso de agentes biológicos como el rituximab surge como nueva terapia de tratamiento tanto para la fase de inducción o recaída, como para el mantenimiento de la enfermedad [15][16][17] . Desde el año 2010 es una opción en inducción o mantenimiento cada vez más atractiva debido a su perfil de seguridad 29,[64][65][66][67][68][69] .…”