Vaculitis Flashcards

1
Q

How does primary vasculitis arise?

A

Inflammatory response (can be autoimmune) that occurs in the walls of blood vessels, causing damage and reduced blood flow

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2
Q

List the 2 main large vessel vasculitis

A

Giant cell arteritis

Takayasu arteritis

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3
Q

List the 2 main medium vessel vasculitis

A

Polyarteritis nodosa

Kawasaki disease

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4
Q

Small vessel vasculitis can be generally divided into which 2 groups?

A

ANCA-associated (+)

Non-ANCA-associated (-)

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5
Q

Takayasu arteritis is commoner in males and in Asian populations. True/False?

A

False

Commoner in females and Asian populations

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6
Q

Giant cell arteritis affects those over 50 and is associated with which other disease?

A

Polymyalgia rheumatica

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7
Q

What is the most common finding in giant cell arteritis?

A

Carotid bruit

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8
Q

What is the mainstay of treatment for large vessel vasculitis?

A

Steroid (40-60mg prednisolone)

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9
Q

Which group of people is Kawasaki disease seen in?

A

Children

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10
Q

Which part of the artery is usually affected in polyarteritis nodosa?

A

Vessel bifurcations

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11
Q

Which hepatitis is associated with polyarteritis nodosa?

A

Hepatitis B

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12
Q

What are the old names for the ANCA-positive small vessel vasculitic diseases?

A

Wegener’s granulomatosis
Churg-Strauss syndrome
Microscopic polyangitis

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13
Q

What are the new names for the ANCA-positive small vessel vasculitic diseases?

A

Granulomatosis with polyangitis (GPA)
Eosinophilic granulomatosis with polyangitis (EGPA)
Microscopic polyangitis (MPA)

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14
Q

Which of the 3 main ANCA-positive small vessel vasculitic diseases involve granulomatous inflammation?

A

GPA

EGPA

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15
Q

Which ANCA-positive small vessel vasculitic disease is associated with asthma?

A

EGPA

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16
Q

Which ANCA-positive small vessel vasculitic diseases are associated with glomerulonephritis?

A

GPA

MPA

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17
Q

GPA is more common in males. True/False?

A

True

18
Q

When does GPA typically present?

A

35-55yrs old

19
Q

What effect does GPA have on the nose?

A

Saddle nose due to cartilage ischaemia

20
Q

What are the other ENT manifestations of GPA?

A
Palpable purpura
Sinusitis
Crusts
Nose bleeds
Deafness
21
Q

What is mononeuritis multiplex and what effect can it have in GPA?

A

Impaired blood supply to nerve

Wrist/foot drop

22
Q

What is the main difference between GPA and EGPA?

A

Late onset asthma

High eosinophil count

23
Q

What are the 2 main types of ANCA?

A

pANCA

cANCA

24
Q

Which vasculitis is associated with pANCA?

A

MPA

EGPA

25
Q

Which vasculitis is associated with cANCA?

A

GPA

26
Q

What is the antigen that pANCA primarily responds to in MPA?

A

MPO

27
Q

What is the antigen that cANCA primarily responds to in GPA?

A

PR3

28
Q

Outline treatment for localised/early systemic immune AAV

A

Methotrexate + steroid

29
Q

Outline treatment for generalised/systemic immune AAV

A

Cyclophosphamide + steroid
Ritixumab + steroid
Azathioprine

30
Q

Outline treatment for refractory AAV

A

IV immunoglobulins

Rituximab

31
Q

What is the main non-ANCA-associated small vessel vasculitic disease?

A

Henoch-Schonlein Purpura (HSP)

32
Q

Which Ig mediates HSP?

A

IgA

33
Q

HSP is most common in which age range?

A

2-11 yrs old

34
Q

Which vessels are usually affected by large vessel vasculitis?

A

Aorta and its branches

35
Q

Giant cell arteritis usually occurs before the age of 50. True/False?

A

False

36
Q

What is the main treatment of ANCA +ve vasculitic disease?

A

IV steroids

Cyclophosphamide

37
Q

How does secondary vasculitis arise?

A
Infection
Drugs
Toxin
Inflammatory disorder
Cancer
38
Q

What respiratory symptom is common in ANCA + vasculitis?

A

Haemoptysis

39
Q

List investigations used in vasculitis

A
Immunofluorescence (cANCA vs pANCA)
CXR
Urinalysis
Bloods - inflammatory markers, U+Es
Biopsy - skin/ kidney
40
Q

List the clinical signs of HSP

A
Purpuric rash (mostly over butt and lower limbs)
Colicky abdominal pain
Bloody diarrhoea
Joint pain and swelling
Renal involvement
41
Q

HSP is a self-limiting disease. True/ False?

A

True

Typically resolves in 8 weeks

42
Q

What is HSP commonly triggered by?

A

URTI/ throat/ GI infections