Medium-vessel vasculitis: Polyarteris Nodusa, Kawasaki disease Flashcards
Is Polyarteritis Nodosa a type of granulomatous or non-granulomatous vasculitis?
Granulomatous
Define necrotising vasculitis?
Vessel wall necrosis due to neutrophil infiltration
Is Polyarteritis Nodosa a type of necrotising or non-necrotising vasculitis?
Necrotising
Which arteries are characteristically affected in Polyarteritis Nodosa, and which circulatory system is not affected?
Renal and visceral (branch of aorta, to internal organs)
arteries
Doesn’t affect pulmonary circulation
What is the typical onset age range for Polyarteritis Nodosa?
40-50
Is Polyarteritis Nodosa equally common in men and women?
Twice as common in men
What is the correlation between Polyarteritis Nodosa and hepatitis B,C?
Up to ⅓ patients have hepatitis B, hepatitis C is less common
What systemic symptoms does Polyarteritis Nodosa present with?
fever, malaise, weight loss, arthralgia, myalgia
Why does Polyarteritis Nodosa present with multi-system symptoms?
Because vasculitis is scattered
Due to involvement of visceral arteries
What 4 skin lesions characteristically present in Polyarteritis Nodosa?
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)
What are infarcts, which present in Polyarteritis Nodosa?
purple or black patches or blood-filled blisters that are dead areas of skin due to blocked blood vessels
What are palpable purpura, which present in Polyarteritis Nodosa?
many red dots on skin which form when there is small vessel vasculitis
What are livedo reticularis, which present in Polyarteritis Nodosa?
red-blue web-like discolouration
Why can Polyarteritis Nodosa commonly cause neuropathy?
In most cases, vasa nervorum (small arteries of peripheral nerves) are affected
How and why does Polyarteritis Nodosa cause hypertension?
Involvement of kidneys causes rapid, severe hypertension
What part of the kidneys is characteristically unaffected by Polyarteritis Nodosa?
Glomeruli
What are the 2 characteristic findings on an angiogram, in Polyarteritis Nodosa?
Multiple small aneurysms
Mesenteric, hepatic, renal artery narrowing
What is the characteristic finding from medium-vessel artery biopsy, in Polyarteritis Nodosa?
Segmental (some areas unaffected) fibrinoid necrosis (necrosis of small vessels)
What medications are generally prescribed to treat Polyarteritis Nodosa?
High-dose glucocorticoids and immunosuppressants
Does Polyarteritis Nodosa have a high or low 5-year survival rate?
High (80%)
If untreated, what is the prognosis of Polyarteritis Nodosa?
Fatal
Is relapse common in treated Polyarteritis Nodosa?
Relapse in 25% cases
Define systemic vasculitis?
Affects multiple areas of the body at the same time
Is Polyarteritis Nodosa a type of systemic vasculitis?
Yes
Is Kawasaki disease characteristically acute (short-term) or chronic (long-term) vasculitis?
Acute
Is Kawasaki disease a type of systemic vasculitis?
Yes
Which arteries are characteristically affected in Kawasaki disease?
Coronary vessels
During which life stages does Kawasaki characteristically present, and what is the typical onset age range?
Infancy and childhood
5 years old and younger
In which country is Kawasaki disease most prevalent?
Japan
In which ethnicity is Kawasaki disease most prevalent?
East-Asian descent
What is the most common pathophysiology of Kawasaki disease?
Immune response to infection (mostly viral)
What skin condition commonly presents in Kawasaki disease, and in which 4 places?
Desquamative/generalised rash (peeling skin looks like scales)
on hands, feet, palms, soles
Which systemic symptom commonly presents in Kawasaki disease, and why?
Fever
To fight infection
How is oral mucosa characteristically affected by Kawasaki disease?
Inflamed and erythema
How are eyes characteristically affected by Kawasaki disease?
Conjunctivitis (red eyes)
Are cardiovascular complications common in Kawasaki disease, and give examples?
20% untreated patients develop cardiovascular complications
eg. myocardial infarction, transient coronary dilatation, myocarditis, pericarditis, peripheral vascular insufficiency and gangrene
What is the gold-standard investigation for Kawasaki disease?
No single gold-standard test, diagnosis is made by ruling out other conditions
What 2 medications are used to treat Kawasaki disease?
Intravenous Immunoglobulin Infusions (IVIs) of 400 mg/kg daily for 4 days to suppress inflammation
Aspirin 5 mg/kg daily for 14 days
How long does it take for someone to make a full recovery from Kawasaki disease when treated, and what complication must they not develop?
Will make full recovery within 6-8 weeks
If they don’t develop aortic aneurysm
If someone with Kawasaki disease develops cardiovascular complications, will they make a full recovery?
No
Will need life-long monitoring
