Vasculitis: Churg-Strauss, Microscopic polyangitis, Polysterteritis Nodosa

Question 1

What is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

what is it associated with?

what does it mostly affect

Answer 1

Small-medium vessel vasculitis
Associated with p-ANCA

mostly affects lungs and skin, can affect other organs

Question 2

What are the features of eosinophilic granulomatosis with polyangiitis?

Answer 2

1. severe Asthma
2. p-ANCA
3. Elevated blood eosinophils in FBC
4. sinusitis
5. dyspnoea

Question 3

How does eosinophilic granulomatasosis with polyangiitis commonly present and when?

Answer 3

with severe asthma in late teenage years or adulthood

Question 4

Which symptoms of eosinophilic granulomatosis with polyangiitis is shared with granulomatosis with polyangiitis (wegner’s granulomatosis)

what is seen in wegner’s that isnt seen in EGwPA

Answer 4

• sinusitis
• dyspnoea

unique to wegner’s

• renal failure
• epistaxis/haemoptysis
• cANCA

Question 5

What may precipitate EGwPA?

Answer 5

Leukotreine receptor antagonists

Question 6

Which ANCA is associated with EGwPA?

Answer 6

pANCA

Question 7

What is microscopic polyngiitis?

which ANCA is it associated with?

Answer 7

• small vessel necrotising vasculitis

- associated more with p-ANCA (majority), 40% c-ANCA

Question 8

What is the main feature of microscopic polyangiits?

what else can it cause?

Answer 8

rapidly progressive renal impairment (glomerulonephritis) - raised creatinine, haematuria, proteinurea

• can cause systematic symptoms e.g lethargy, myalgia, weight loss
• can cause cough, dyspnoea and haemoptysis

Question 9

What is polyarteritis nodosa?

what can it lead to?

Answer 9

• medium vessel necrotising vasculitis, pANCA associated

- necrotising inflammation leads to aneurysm formation and can lead to infarction

Question 10

Which demographic is polyarteritis nodosa commonest in?

Answer 10

Middle aged men

Question 11

What is polyarteritis nodosa associated with?

Answer 11

Hepatitis B infection

Question 12

What is a key feature of Polyarteritis Nodosa?

What are other features of polyarteritis nodosa?

Answer 12

1. Livedo reticularis - mottled skin rash
2. systemic symptoms - fever, malaise, arthralgia
3. renal failure, haematuria
4. HTN, MI

Question 13

What is the management of polyarteritis nodosa

Answer 13

manage htn

steroids for mild cases