Pulmonary vasculitis

Question 1

what is eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?

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. Affected people may have perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) directed against myeloperoxidase (MPO). These autoantibodies are more common in patients with more vasculitic manifestations, such as glomerulonephritis.

Question 2

what is Granulomatosis with polyangiitis?

Answer 2

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. Involvement of cutaneous, ocular, musculoskeletal, and peripheral nervous system tissue is also common.
CANCA antibodies
epithelial crescents in bowman’s space

Question 3

what is the stereotypical history of someone with granulomatosis with polyangitis?

Answer 3

a 50-year-old man with a history of sinusitis presents with haemoptysis and renal impairment. A renal biopsy shows crescentic glomerulonephritis. saddle shaped nose deformity
A 45-year-old man is investigated for renal impairment. Over the past few weeks he has suffered from chronic sinusitis and haemoptysis

Question 4

what type of vaculitis are granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis?

Answer 4

small vessel, ANCA-associated vasculitis