Introduction to AAV

AAV is a rare, severe small vessel vasculitis that affects multiple organs and has a high acute mortality risk.1

Incidence & prevalence of AAV

AAV is a rare disease2

  • Overall annual incidence rate in Europe: Approximately 13–20 cases per million2
  • Prevalence: Approximately 46–184 cases per million people2,3

AAV occurrence

  • AAV can affect both younger and older people, but is rare in children and young people and incidence rises with age2,4
  • AAV occurs slightly more frequently in men (annual incidence rate approximately 60%) than in women2,4

GPA (Granulomatosis with Polyangiitis, previously called Wegener’s) and MPA (Microscopic Polyangiitis) are the most common clinical subtypes of AAV2

Anca-associated vasculitis types incidence on map of Europe. GPA is most predominant in Northern Europe, MPA in Southern Europe.

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 (Eosinophilic Granulomatosis with Polyangiitis, previously called Churg-Strauss syndrome) 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 underlines the importance of early diagnosis in order to improve renal outcomes.5†

Schematic diagram of areas affected by vasculitis


GPA: 60–80%; MPA: 80%; EGPA: 20%

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

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 current 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*

Graph of how ANCA-associated vascultis patients are referred to a specialist. 25% go directly, but 75% are referred by another physician.

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*

Vasculitis patient journey referral diagnosis followup

  • Referral
  • Diagnosis
  • Follow-up
Vasculitis patient journey referral mobile

References & footnotes




EGPA is distinctly different from GPA and MPA1

EGPA (Eosinophilic Granulomatosis with Polyangiitis, previously called Churg-Strauss syndrome) 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

Areas affected by vasculitis diagram schema full Areas affected by vasculitis diagram chart

References & footnotes