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Treatment pathway

AAV is a chronic condition with a relapsing course that needs long-term immunosuppressive therapy1–4

EULAR recommendations state that AAV patients should be offered best care which must be based on shared decision-making between the patient and the physician, considering efficacy, safety and costs5

AAV Treatment Graph

Diagram adapted from Hellmich B. Update of the EULAR Recommendations on the Management of ANCA-associated Vasculitis. Presented at EULAR 2022. Full guidelines not yet published. Always refer to the product SmPC for approved indication before prescribing

AAV Treatment Graph

Diagram adapted from Hellmich B. Update of the EULAR Recommendations on the Management of ANCA-associated Vasculitis. Presented at EULAR 2022. Full guidelines not yet published. Always refer to the product SmPC for approved indication before prescribing

The balance of treatment

Treatment must balance controlling vasculitis with minimising treatment-related damage1–4

At the time of AAV diagnosis or relapse, the major concerns around treatment involve controlling vasculitis activity and minimising the acute adverse effects from therapy.1,2 Later when remission is achieved, cumulative organ damage, often related to long-term low dose glucocorticoids, and patient experience are more important.1–4

The EULAR Guidelines now state that patients should be periodically screened for treatment-related adverse effects and co-morbidities. They recommend prophylaxis and life-style advice to reduce treatment-related complications and other co-morbidities.5

References & footnotes

Treatment-related AEs are the leading cause of acute mortality1

In the first year after a GPA or MPA diagnosis, 56 of 524 patients died. A total of 59% of these deaths were as a result of treatment-related AEs.1*

A systematic literature review of glucocorticoid-related AEs in AAV clinical studies published between 1 January 2007 and 30 January 2018 identified mortality (33%) as the glucocorticoid-related serious AE with the highest frequency observed during early induction treatment. Other common glucocorticoid-related serious AEs experienced by patients include infections (20%), musculoskeletal (17%) and renal disorders (15%).2†

References & footnotes
Treatment-related

Latest clinical data in AAV

RAVE

Rituximab (RTX) was found to be as effective as cyclophosphamide (CYC) for the induction and maintenance of remission, but remission rates remain variable

2010

RITAZAREM

Preliminary results show that a reduced glucocorticoid (GC) dose can be effective for reinducing remission in AAV. RTX was superior to azathioprine (AZA) at maintaining remission

2014

PEXIVAS

Reducing the GC dose in severe AAV patients did not significantly impact the primary outcome of death or end-stage renal disease, but did reduce the rate of infection (HR=0.69)

2018

ADVOCATE

Avacopan-based regimen is
non-inferior at achieving remission at Week 26 and superior at sustaining remission at Week 52 compared to a GC-based regimen1

2018

2010

2014

2018

2019

2020

2021

MAINRITSAN 1

RTX is more effective than AZA for maintenance of remission, but GC use and relapse remain common

2019

MAINRITSAN 2

Tailored and fixed-schedule RTX regimens are equally as effective at maintaining remission, but relapses still occur

2020

MAINRITSAN 3

Extended maintenance therapy leads to better clinical outcomes

2020

Real-World Experience

Many patients do not achieve or sustain full remission1*

Patient response to remission induction therapy is variable. After 12 months of therapy (n=929), 59% of GPA (granulomatosis with polyangiitis, previously called Wegener’s) and MPA (microscopic polyangiitis) patients achieve full remission, and >50% of patients continue glucocorticoid use.1 More patients need to achieve and sustain remission without prolonged glucocorticoid use.

image1
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References & footnotes

Real-world treatment data demonstrates that AEs remain an issue for new and relapsing patients1,2

Over the first 12 months of treatment in patients with GPA or MPA, AEs are common.1,2 At this time, glucocorticoid dose is typically highest, as current treatment guidelines recommend the use of glucocorticoids for remission induction.1–3 The majority of patients continue to use glucocorticoids 12 months after starting remission induction.1,2

New patients (n=929)1*

Graph showing frequency of Adverse events in new patients month by month.Graph showing frequency of Adverse events in new patients month by month.Graph showing frequency of Adverse events in new patients month by month.Graph showing frequency of Adverse events in new patients month by month.
 
 
 
 

Relapsing patients (n=268)2*

Graph showing frequency of Adverse events in relapsing patients month by month.Graph showing frequency of Adverse events in relapsing patients month by month.Graph showing frequency of Adverse events in relapsing patients month by month.Graph showing frequency of Adverse events in relapsing patients month by month.
 
 
 
 
Introduction to AAV

Introduction to AAV

AAV is a rare, severe small vessel vasculitis that affects multiple organs and has a high acute mortality risk1

Read more
Disease Mechanism

Disease mechanism

The interaction between the activated alternative complement pathway, neutrophilsand C5a is at the heart of vasculitic damage in AAV2

Read more
AAV treatment

AAV treatment

A framework of practice for the treatment and management of AAV patients

Read more
References & footnotes
EUVAS

European Vasculitis Society website

Read more
ASN

American Society of Nephrology

Read more
ACR

American College of Rheumatology

Read more
ERA

European Renal Association

Read more
EULAR

European Alliance of Associations for Rheumatology

Read more
KDIGO

Kidney Disease Improving Global Outcomes

Read more
KDIGO recommendations

KDIGO recommendations for the management of individuals with glomerular diseases

Read more
EULAR treatment recommendations

EULAR recommendations for the management of ANCA-associated vasculitis

Read more
EULAR recommendations pocket guide

Summary of EULAR recommendations for the management of GPA/MPA

Download
Patient videos and quality of life
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Vasculitis International

Europe

www.vasculitisint.com
Selbsthilfe Vaskulitis e.V.

Germany

www.vasculitisint.com/vpag/vaskulitis-e-v
Associazione Pazienti della Sindrome di Churg Strauss

Italy

www.apacs-egpa.org
Suomen Vaskuliittiyhdistys ry

Finland

www.vaskuliittiyhdistys.fi
Selbsthilfe Vaskulitis Mainz

Germany

vaskulitis-mainz@gmx.de
Vasculitis Stichting

Netherlands

www.vasculitis.nl
Liga Portuguesa Contra as Doencas Reumaticas

Portugal

www.lpcdr.org.pt
Association Vascularites

France/Belgium

www.association-vascularites.org

Ireland

Association Espanola de Vasculitis Sistemicas

Spain

www.sid-inico.usal.es

test

Associazione Malattie Autoimmuni

Italy

www.associazionemalattieautoimmuni.it
Associazione Nazionale Malati Reumatici ONLUS

Italy

www.anmar-italia.it
Australia and New Zealand Vasculitis Society

Australia / New Zealand

www.anzvasculitis.org
Federación Española de Enfermedades Raras

Spain

www.enfermedades-raras.org
Liga Reumatológica Española

Spain

lire.es/
Vasculitis Stowarzyszenie Chorych

Poland

vasculitis.org.pl/