Vasculitides - Granulomatosis with Polyangiitis

Question 1

Define granulomatosis with polyangiitis.

Answer 1

Granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) is a systemic vasculitis that typically involves small and medium vessels. Although any organ may be targeted, the classic triad consists of upper and lower respiratory tract involvement and pauci-immune glomerulonephritis.

Question 2

Explain the aetiology/risk factors of granulomatosis with polyangiitis.

Answer 2

Genetic predisposition
Infection
Environmental exposures
All of these have a weak association, though.

Question 3

Summarise the epidemiology of granulomatosis with polyangiitis.

Answer 3

The incidence and prevalence of granulomatosis with polyangiitis (GPA) (formerly known as Wegener’s granulomatosis) varies considerably between countries. GPA can occur at any age. The mean age of onset in most series is between 40 and 60 years of age, with an approximately equal gender distribution. It is most commonly seen in white people, but can also occur in other racial and ethnic groups.

Question 4

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

Answer 4

Upper and lower respiratory tract involvement
Renal involvement
Constitutional features
Ocular manifestations
Cutaneous manifestations
Musculoskeletal manifestations
Neurological manifestations

Question 5

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

Answer 5

Urinalysis and microscopy
CT chest
Anti-neutrophil cytoplasmic antibody (ANCA)
FBC and differential
Serum creatinine
Erythrocyte sedimentation rate (ESR)