Current therapies

Treatment has improved, but treatment-related complications remain common.

Treatment pathway


AAV is a long-term condition with a relapsing
course that needs long-term immunosuppresive therapy1-3

  • Organ- or life-threatening disease
  • Remission
  • Relapse
  • Refractory
Vasculitis treatment pathway of organs or life threatening disease chart Vasculitis treatment pathway of organs or life threatening disease mobile chart

Treatment-related infection is the leading cause of acute mortality1,2


Treatment-related infection is the
leading cause of acute mortality1,2

Vasculitis causes of mortality chart

In the first year after a GPA or MPA diagnosis, 50% of the early mortality risk is due to treatment-related infection1*

A systematic literature review of glucocorticoid-related AEs in AAV clinical studies published between 1 January 2007 and 30 January 2018 identified infection as the leading cause of mortality, with the highest frequency observed during early induction treatment.2†

Real-world data confirm that treatment-related infections are common with 27%, 28%, 23% and 20% of GPA and MPA patients experiencing an infection at 1, 3, 6 and 12 months following the commencement of remission induction therapy, respectively.3‡

References & footnotes

Organ damage in AAV


GPA and MPA patients accumulate organ damage from a
combination of vasculitis activity and glucocorticoid-related AEs1–3

Long-term and repeated high-dose glucocorticoid use is associated with an increased risk of new onset/worsening of diabetes mellitus, hypertension, osteoporosis, avascular necrosis of bone, malignancy, cataracts and other debilitating side effects.1,2*† In a study following newly diagnosed GPA and MPA patients for up to 7 years, the frequency of damage, including potentially treatment-related damage, rose over time (p<0.01).2†

High levels of long-term vasculitis damage were independently associated with increased cumulative glucocorticoid use (p=0.016).3†

This serious morbidity translates into a significantly increased mortality risk, with a hazard ratio of 2.41 (95% CI: 1.74–3.34) in GPA patients compared with age- and sex-matched controls.2–4†‡

Vasculitis organ damage chart
References & footnotes

New therapies and innovative strategies are needed1,2


Lightbulb icon

"Ongoing advances in understanding ANCA disease
mechanisms, and development of more effective, less toxic, and more targeted therapies, undoubtedly will lead to even better outcomes in the future."3

– Jennette JC, Nachman PH. Clin J Am Soc Nephrol 2017


Syringe icon

"Biologic therapies that target specific cellular and molecular components of the autoimmune response and the mediators of inflammatory injury may be more effective and less toxic."3

– Jennette JC, Nachman PH. Clin J Am Soc Nephrol 2017

References & footnotes

Introduction to AAV

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

Vasculitis artwork pathway torso

Disease mechanism

The interaction between the activated alternative complement pathway, neutrophils and C5a is at the heart of vasculitic damage in AAV

Vasculitis artwork pathophysiology

Current therapies

Treatment has improved but treatment-related complications remain common

Vasculitis artwork kidneys

Patient experience

AAV is an emotional journey from the patient's perspective

Vasculitis artwork patient portrait

Investigate AAV

Take the interactive challenge to assess your understanding of AAV. Fully interactive version available on desktop only

Vasculitis artwork lungs