Introduction to AAV

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

Incidence & prevalence of AAV


AAV is a rare disease1

  • Overall annual incidence rate in Europe: Approximately 13–20 cases per million1
  • Prevalence: Approximately 46–184 cases per million people1,2

AAV occurrence

  • AAV can affect both younger and older people, but is rare in children and young people and incidence rises with age1,3
  • AAV occurs slightly more frequently in men (annual incidence rate approximately 60%) than in women1,3
References & footnotes

Systemic organ damage


AAV can result in systemic organ damage and failure, with the kidney and lung as major targets1,2

AAV can result in damage to vital organs such as the lungs, kidneys, nervous system, gastrointestinal system, skin, eyes and heart.3 Both GPA and MPA show a lot of clinical commonality, whereas EGPA is distinctly different.2

As many as 23% of PR3- and MPO-ANCA positive patients who require RRT at the time of diagnosis die within 6 months and 29% do not regain renal function.4* In patients with renal involvement at diagnosis, ESRD occurs in 13% of PR3- and MPO-ANCA positive patients within 3 years of diagnosis.5† Compared with a short prodromal phase, patients with a long prodromal phase (>22 weeks between first AAV symptoms and diagnosis) are more likely to have proteinuria present at 6 months and are at double the risk of 3-year ESRD, which underlies the importance of early diagnosis in order to improve renal outcomes.5†

Kidneys2

Frequency
GPA: 60–80%; MPA: 80%; EGPA: 20%

Manifestations
GPA, MPA and EGPA: Pauci-immune
necrotising extra-capillary
glomerulonephritis

References & footnotes

Mortality & morbidity


AAV leads to an increased risk of mortality, especially in the first year after diagnosis1*

In the first year after a GPA or MPA diagnosis
(n=524), the mortality rate is 10.7% (n=561).1*

Of these patients

Half (50%) of mortality in the first year
is due to treatment-related infection.1

In clinical trial settings, the cumulative survival rate at 2 and 5 years in patients with newly diagnosed GPA and MPA is 85% and 78%, respectively.2†

Population-based data suggest that the long-term mortality rate in patients with AAV has improved considerably over the past two decades, but is still not as good as matched controls.3‡

In a study following individuals for a median 5.2 years, the mortality rate in incident GPA or MPA patients receiving conventional treatment was 2.6 times higher than an age- and sex-matched general population (p<0.0001).2†

References & footnotes

Referral, diagnosis & follow-up


AAV patients often experience a complex pathway of patient referral and diagnosis1*

Many patients have renal disease at presentation, but general non-specific referral symptoms predominate1

Renal disease: 64%

Fatigue: 58%

Fever: 54%

Weight loss: 53%

Joint pain: 47%

16% of patients have had their referral symptoms for over 3 months before receiving an AAV diagnosis1

Comorbidities at diagnosis are common (65% of patients)1

Hypertension: 45%

Type 2 diabetes: 16%

COPD/asthma: 15%

Coronary arterial disease: 10%

Arthritis: 9%

Osteoporosis: 7%

BMI >35: 6%

Cardiac failure: 6%

The relative rarity and non-specific presentation of AAV can lead to a delay in disease diagnosis of more than 6 months in one-third of patients.2

Diagnosis of AAV and differentiation into the GPA, MPA or EGPA subtype depends on the patient’s clinical symptom constellation, the results of imaging studies, and laboratory investigation.2,3

Due to the linkage of anti-PR3 with GPA and anti-MPO with MPA, ANCA testing is critical for diagnosis.2–6

  • Up to 20% of GPA and MPA patients and over 60% of EGPA patients are ANCA negative3

  • A positive ANCA test result can be found in other conditions, e.g. autoimmune hepatitis, ulcerative colitis, infection with hepatitis C virus or HIV, or infectious endocarditis, without associated vasculitis3

An organ biopsy, usually renal, is often performed to confirm the diagnosis.


AAV patients often experience a complex pathway of patient referral and diagnosis1*

  • Referral
  • Diagnosis
  • Follow-up
References & footnotes

EGPA


EGPA is distinctly different from GPA and MPA1

EGPA is rarer than either GPA or MPA.2 In patients with EGPA, peripheral neuropathy is very frequent and almost all patients have asthma. ENT manifestations are frequent, but the kidneys are not commonly involved. In EGPA, ANCA are most commonly directed against MPO. However, over 60% of EGPA patients are ANCA negative.1

These individuals may have more frequent cardiac involvement, with less renal or peripheral nerve contribution.1 The absence of granuloma helps to differentiate MPA from GPA and EGPA.1,3 The presence of granuloma, the symptoms experienced, the organs involved and the type of ANCA differentiate GPA from EGPA.1,3

References & footnotes

Introduction to AAV

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

Disease mechanism

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

Current therapies

Treatment has improved but treatment-related complications remain common

Patient experience

AAV is an emotional journey from the patient's perspective

Investigate AAV

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