2020
DOI: 10.1136/bmj.m421
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Management of ANCA associated vasculitis

Abstract: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a small to medium vessel vasculitis associated with excess morbidity and mortality. This review explores how management of AAV has evolved over the past two decades with pivotal randomized controlled trials shaping the management of induction and maintenance of remission. Contemporary AAV care is characterized by approaches that minimize the cumulative exposure to cyclophosphamide and glucocorticoids, increasingly use rituximab for remi… Show more

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Cited by 63 publications
(71 citation statements)
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“…Notably, cost estimation reported by us represents the first data available in the literature coming from a European healthcare system. The management of AAV has evolved considerably in the recent decades, allowing improvements in outcomes and overall survival [19][20][21][22]. Nevertheless, the disease itself and, importantly, superimposed infections are still the main causes of hospitalization in AAV [3,23].…”
Section: Discussionmentioning
confidence: 99%
“…Notably, cost estimation reported by us represents the first data available in the literature coming from a European healthcare system. The management of AAV has evolved considerably in the recent decades, allowing improvements in outcomes and overall survival [19][20][21][22]. Nevertheless, the disease itself and, importantly, superimposed infections are still the main causes of hospitalization in AAV [3,23].…”
Section: Discussionmentioning
confidence: 99%
“…The success story of rituximab in the management of ANCA-associated vasculitis has been reviewed in detail elsewhere [ 169 ]. Both seminal studies, RAVE and RITUXVAS [ 64 , 65 ], showed a non-inferiority of rituximab in the induction of remission compared to a cyclophosphamide-based induction therapy.…”
Section: Therapeutic Implications Of Biomarker Discoveriesmentioning
confidence: 99%
“…A non-significant increase in malignancies has been reported in RAVE in the rituximab arm. Real-life data from a single-expert center found that malignancy risk following rituximab is not increased during the follow-up period [ 169 , 170 ]. Following a disease relapse, rituximab showed superiority compared to a cyclophosphamide-based induction therapy in achievement of remission [ 64 ].…”
Section: Therapeutic Implications Of Biomarker Discoveriesmentioning
confidence: 99%
“…In such cases the use of GCs and biological drugs (anti-IL6, anti-IL1, JAK inhibitors) could be effective. GCs are often used in vasculitis with severe organ involvement [100]. Patients with kidney and/or pulmonary involvement (i.e.…”
Section: Exogenous Cushing's Syndrome Due To Glucocorticoid Administrmentioning
confidence: 99%
“…Patients with kidney and/or pulmonary involvement (i.e. ANCA-associated vasculitis) and with concomitant use of immunosuppressive drugs could present more rapid clinical progression of COVID-19 infection, similarly to transplant patients [100,101]. There are no studies to date showing a higher incidence of rheumatological diseases in patients with COVID-19 infection.…”
Section: Exogenous Cushing's Syndrome Due To Glucocorticoid Administrmentioning
confidence: 99%