Medium-vessel vasculitis: Polyarteris Nodusa, Kawasaki disease

Question 1

Is Polyarteritis Nodosa a type of granulomatous or non-granulomatous vasculitis?

Answer 1

Granulomatous

Question 2

Define necrotising vasculitis?

Answer 2

Vessel wall necrosis due to neutrophil infiltration

Question 3

Is Polyarteritis Nodosa a type of necrotising or non-necrotising vasculitis?

Answer 3

Necrotising

Question 4

Which arteries are characteristically affected in Polyarteritis Nodosa, and which circulatory system is not affected?

Answer 4

Renal and visceral (branch of aorta, to internal organs)
arteries

Doesn’t affect pulmonary circulation

Question 5

What is the typical onset age range for Polyarteritis Nodosa?

Answer 5

40-50

Question 6

Is Polyarteritis Nodosa equally common in men and women?

Answer 6

Twice as common in men

Question 7

What is the correlation between Polyarteritis Nodosa and hepatitis B,C?

Answer 7

Up to ⅓ patients have hepatitis B, hepatitis C is less common

Question 8

What systemic symptoms does Polyarteritis Nodosa present with?

Answer 8

fever, malaise, weight loss, arthralgia, myalgia

Question 9

Why does Polyarteritis Nodosa present with multi-system symptoms?

Answer 9

Because vasculitis is scattered

Due to involvement of visceral arteries

Question 10

What 4 skin lesions characteristically present in Polyarteritis Nodosa?

Answer 10

Ulcerations

Infarcts (purple or black patches or blood-filled blisters that are dead areas of skin due to blocked blood vessels)

Palpable purpura (many red dots on skin which form when there is small vessel vasculitis)

Livedo reticularis (red-blue web-like discolouration)

Question 11

What are infarcts, which present in Polyarteritis Nodosa?

Answer 11

purple or black patches or blood-filled blisters that are dead areas of skin due to blocked blood vessels

Question 12

What are palpable purpura, which present in Polyarteritis Nodosa?

Answer 12

many red dots on skin which form when there is small vessel vasculitis

Question 13

What are livedo reticularis, which present in Polyarteritis Nodosa?

Answer 13

red-blue web-like discolouration

Question 14

Why can Polyarteritis Nodosa commonly cause neuropathy?

Answer 14

In most cases, vasa nervorum (small arteries of peripheral nerves) are affected

Question 15

How and why does Polyarteritis Nodosa cause hypertension?

Answer 15

Involvement of kidneys causes rapid, severe hypertension

Question 16

What part of the kidneys is characteristically unaffected by Polyarteritis Nodosa?

Answer 16

Glomeruli

Question 17

What are the 2 characteristic findings on an angiogram, in Polyarteritis Nodosa?

Answer 17

Multiple small aneurysms

Mesenteric, hepatic, renal artery narrowing

Question 18

What is the characteristic finding from medium-vessel artery biopsy, in Polyarteritis Nodosa?

Answer 18

Segmental (some areas unaffected) fibrinoid necrosis (necrosis of small vessels)

Question 19

What medications are generally prescribed to treat Polyarteritis Nodosa?

Answer 19

High-dose glucocorticoids and immunosuppressants

Question 20

Does Polyarteritis Nodosa have a high or low 5-year survival rate?

Answer 20

High (80%)

Question 21

If untreated, what is the prognosis of Polyarteritis Nodosa?

Answer 21

Fatal

Question 22

Is relapse common in treated Polyarteritis Nodosa?

Answer 22

Relapse in 25% cases

Question 23

Define systemic vasculitis?

Answer 23

Affects multiple areas of the body at the same time

Question 24

Is Polyarteritis Nodosa a type of systemic vasculitis?

Answer 24

Yes

Question 25

Is Kawasaki disease characteristically acute (short-term) or chronic (long-term) vasculitis?

Answer 25

Acute

Question 26

Is Kawasaki disease a type of systemic vasculitis?

Answer 26

Yes

Question 27

Which arteries are characteristically affected in Kawasaki disease?

Answer 27

Coronary vessels

Question 28

During which life stages does Kawasaki characteristically present, and what is the typical onset age range?

Answer 28

Infancy and childhood

5 years old and younger

Question 29

In which country is Kawasaki disease most prevalent?

Answer 29

Japan

Question 30

In which ethnicity is Kawasaki disease most prevalent?

Answer 30

East-Asian descent

Question 31

What is the most common pathophysiology of Kawasaki disease?

Answer 31

Immune response to infection (mostly viral)

Question 32

What skin condition commonly presents in Kawasaki disease, and in which 4 places?

Answer 32

Desquamative/generalised rash (peeling skin looks like scales)

on hands, feet, palms, soles

Question 33

Which systemic symptom commonly presents in Kawasaki disease, and why?

Answer 33

Fever

To fight infection

Question 34

How is oral mucosa characteristically affected by Kawasaki disease?

Answer 34

Inflamed and erythema

Question 35

How are eyes characteristically affected by Kawasaki disease?

Answer 35

Conjunctivitis (red eyes)

Question 36

Are cardiovascular complications common in Kawasaki disease, and give examples?

Answer 36

20% untreated patients develop cardiovascular complications

eg. myocardial infarction, transient coronary dilatation, myocarditis, pericarditis, peripheral vascular insufficiency and gangrene

Question 37

What is the gold-standard investigation for Kawasaki disease?

Answer 37

No single gold-standard test, diagnosis is made by ruling out other conditions

Question 38

What 2 medications are used to treat Kawasaki disease?

Answer 38

Intravenous Immunoglobulin Infusions (IVIs) of 400 mg/kg daily for 4 days to suppress inflammation

Aspirin 5 mg/kg daily for 14 days

Question 39

How long does it take for someone to make a full recovery from Kawasaki disease when treated, and what complication must they not develop?

Answer 39

Will make full recovery within 6-8 weeks

If they don’t develop aortic aneurysm

Question 40

If someone with Kawasaki disease develops cardiovascular complications, will they make a full recovery?

Answer 40

No

Will need life-long monitoring