29th January 2024

Background

Epidemiology

Granulomatosis with polyangiitis, which was formerly known as Wegener’s granulomatosis, affects equal numbers of males and females. The peak incidence is around 40-60 years of age. Prevalence is approximately 3 per 100,000 population.

Aetiology

Idiopathic.

Pathophysiology

Aberrant epigenetic expression of PR-3 on the cell membranes of neutrophils binds to c-ANCA. This activates neutrophils to release neutrophilic inflammatory markers. This process damages the endothelial cells of the small blood vessels.