29th January 2024

Background

Epidemiology 

This disease typically affects middle-aged individuals, with equal distribution of disease amongst males and females. 

Aetiology

Unknown, however, the cause is likely multifactorial with genetic, environmental and infective precipitants. 

Pathophysiology 

Unidentified allergens stimulate an adaptive immune response, leading to a B-cell response and IgG4, IgE and ANCA production. This process guides eosinophils to the tissues and stimulates degranulation of activated eosinophils, causing damage and necrosis of tissues and vessels.