Vasculitis: Wegener's granulomatosis (granulomatosis and polyangiitis)

Question 1

Definition

Answer 1

Vasculitis which most commonly affects
- Arterioles
- Capillaries
- Venules

Question 2

Epidemiology

Answer 2

Middle aged males

Question 3

Pathophysiology

Answer 3

B-cells mistakenly target their antibodies to granules made by a person’s own neutrophils
- The B-cells release an autoantibody called cytoplasmic antineutrophil cytoplasmic antibody or c-ANCA = bind to specific neutrophil granule = proteinase 3 = embedded on membrane of some neutrophils
Once binding causes neutrophil to release oxygen free radicals = indirectly damage nearby endothelial cells

Question 4

Signs

Answer 4

Nasopharynx
- Chronic pain from sinusitis
- Bloody mucus from ulcers
= SADDLE NOSE DEFORMITIY
Lungs
- Airways constrict + ulcer formation = bloody coughing
Kidneys
- Glomerulonephritis
- decreased urine production
- increased blood pressure

Question 5

Treatment

Answer 5

FIRST LINE = PREDNISOLONE