Management of ANCA associated vasculitis

Wallace, Zachary S; Miloslavsky, Eli M.

doi:10.1136/bmj.m421

Cited by 68 publications

(81 citation statements)

References 154 publications

(188 reference 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%

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Quartuccio

Treppo

Valent³

et al. 2020

Intern Emerg Med

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ANCA-associated vasculitides (AAV) comprise a group of systemic vasculitides characterized by inflammation of small-sized blood vessels leading to multi-organ involvement. The worldwide annual incidence of AAV ranges from 1.2 to 3.3 cases per 100 000 individuals with a prevalence of 4.6-42.1 cases per 100 000 individuals. The prevalence of AAV is geographically heterogeneous; therefore, regional epidemiological studies can be more informative to improve health care systems. Even though clinicians are aware that the healthcare burden and the risk of hospitalization of AAV appear high, data on hospitalization and cost of illness due to AAV are still scarce or even lacking. This study aims to characterize the economic burden of AAV in Friuli Venezia Giulia (FVG), Italy. Thus, a retrospective study was conducted through the integration of many administrative health databases of the FVG as the source of information. From data integration, we estimated that more than two-thirds of AAV patients showed at least one hospitalization in their medical history, most frequently caused by the disease itself or superimposed infections. Around 10% of patients developed end-stage renal disease. In an 8-year follow-up, the overall healthcare cost was € 1,215,078, corresponding to € 6,168 patient-year. ANCA-positive patients showed much higher costs than ANCA-negative patients did. Overall, AAV are rare diseases, but imply very high healthcare costs. Early diagnosis and optimal treatment probably still remain unmet needs for AAV.

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Section: Discussionmentioning

confidence: 99%

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Quartuccio

Treppo

Valent³

et al. 2020

Intern Emerg Med

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“…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%

Immunopathogenesis of ANCA-Associated Vasculitis

Kronbichler

Lee

Denicolò

et al. 2020

IJMS

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small- and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.

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“…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%

Glucocorticoid excess and COVID-19 disease

Guarnotta

Ferrigno

Martino

et al. 2020

Rev Endocr Metab Disord

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The pandemic of coronavirus disease (COVID-19), a disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is causing high and rapid morbidity and mortality. Immune system response plays a crucial role in controlling and resolving the viral infection. Exogenous or endogenous glucocorticoid excess is characterized by increased susceptibility to infections, due to impairment of the innate and adaptive immune system. In addition, diabetes, hypertension, obesity and thromboembolism are conditions overrepresented in patients with hypercortisolism. Thus patients with chronic glucocorticoid (GC) excess may be at high risk of developing COVID-19 infection with a severe clinical course. Care and control of all comorbidities should be one of the primary goals in patients with hypercortisolism requiring immediate and aggressive treatment. The European Society of Endocrinology (ESE), has recently commissioned an urgent clinical guidance document on management of Cushing’s syndrome in a COVID-19 period. In this review, we aim to discuss and expand some clinical points related to GC excess that may have an impact on COVID-19 infection, in terms of both contagion risk and clinical outcome. This document is addressed to all specialists who approach patients with endogenous or exogenous GC excess and COVID-19 infection.

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Management of ANCA associated vasculitis

Cited by 68 publications

References 154 publications

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

Immunopathogenesis of ANCA-Associated Vasculitis

Glucocorticoid excess and COVID-19 disease

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