Vasculitides - Eosinophilic Granulomatosis with Polyangiitis

Question 1

Define eosinophilic granulomatosis with polyangiitis.

Answer 1

Eosinophilic granulomatosis with polyangiitis (EGPA) is a form of primary systemic autoimmune vasculitis characterised by inflammation of blood vessels. In EGPA, vasculitis is associated with asthma and eosinophilia. EGPA is also known as Churg-Strauss syndrome (CSS).

Question 2

Explain the aetiology/risk factors of eosinophilic granulomatosis with polyangiitis.

Answer 2

History of asthma, allergic rhinitis, or sinusitis
Use of certain medications

Question 3

Summarise the epidemiology of eosinophilic granulomatosis with polyangiitis.

Answer 3

EGPA is the most rare of the three anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides, with an estimated annual incidence of between 1 and 3 cases per million people. Among asthma patients, the incidence is as high as 67 per million asthma patients.

Question 4

Recognise the presenting symptoms of eosinophilic granulomatosis with polyangiitis. Recognise the signs of eosinophilic granulomatosis with polyangiitis on physical examination.

Answer 4

Focal numbness or weakness
Nasal discharge or stuffiness, or facial pain
Palpable purpura and petechiae
Wheeze
Haemoptysis
Skin nodules
Fatigue, arthralgias, myalgias
Shortness of breath or cough
Abdominal pain

Question 5

Identify appropriate investigations for eosinophilic granulomatosis with polyangiitis and interpret the results.

Answer 5

FBC with differential
Serum anti-neutrophil cytoplasmic antibodies (ANCA)
Serum CRP
Erythrocyte sedimentation rate
Serum urea and creatinine
Urinalysis
Pulmonary function test
Chest x-ray
Echocardiogram