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Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis

A

inflammation of blood vessels

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2
Q

generally, how is vasculitis classified

A

histological type of inflammation
size of vessel segment involved
the distribution of involved vessels

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3
Q

2 types of large vessel vasculitis

A

Giant Cell Arteritis

Takayasu arteritis

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4
Q

2 types of medium vessel vasculitis

A

Kawasaki disease

Polyarteritis nodosa

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5
Q

Types of small vessel vasculitis

A

ANCA-associated

Non- ANCA associated

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6
Q

Types of ANCA-associated small vessel vasculitis

A

Microscopic polyangitis

Granulomatosis with polyangitis (Wegener)

Eosinophilic granulomatosis with polyangitis (Churg-Strauss)

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7
Q

Types of non-ANCA associated small vessel vasculitis

A

Cryoglobulinemic vasculitis

IgA vasculitis (Henoch-Schonlein)

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8
Q

what vessels do Large vessel arteritis affect

A

aorta and its major branches

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9
Q

What is the main difference between Giant Cell arteritis and Takaysu Arteritis

A

Age of onset

GCA occurs rarely before 50 years

TA occurs rarely after 50 years

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10
Q

Describe pathology of GCA

A

transmural inflammation of the intima, media and adventitia of affected arteries.
Patch infiltration by lymphocytes, macrophages, and multinucleated giant cells.
Vessel wall thickening - arterial luminal narrowing, resulting is ischaemia.

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11
Q

condition commonly associated with GCA

A

polymyalgia rheumatica

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12
Q

artery most commonly affected by GCA

A

temporal artery

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13
Q

presentation of GCA

A

visual disturbances
headache
jaw claudication
scalp tenderness (esp with hair combing)

constitutional symptoms:
fatigue
malaise
fever

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14
Q

what symptom of GCA is the most significant causes of morbidity

A

visual disturbances

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15
Q

what causes the jaw claudication seen in GCA

A

ischaemia of the maxillary artery

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16
Q

visual disturbances seen in GCA

A

unilateral visual blurring
unilateral visial loss
painless
diplopia

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17
Q

definitive test for GCA

A

temporal artery biopsy

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18
Q

main problem with temporal artery biopsy as diagnostic test for GCA

A

it has 100% specificity, but only 15-40% sensitivity (patchy involvement of artery, so some segments may be normal)

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19
Q

typical biopsy findings in GCA

A

mononuclear infiltration or granulomatous inflammation with multinucleated giant cells

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20
Q

Mx of GCA

A

40mg pred if no visual symptoms

60mg pred if visual symptoms

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21
Q

when should treatment of GCA be started

A

as soon as there is clinical suspicion - dont wait for biopsy!!

22
Q

inflammatory markers in GCA

A

plasma viscosity
ESR
CRP
- all elevated

23
Q

most common group of people to get Takayasu Arteritis

A

females <40 years and Asian populations

24
Q

Consequence of untreated large vessel vasculitis

A

vascular stenosis and aneurysms

- results in reduced pulses and (carotid) bruits

25
Q

Tx Takayasu Arteritis

A

same as for GCA

40-60mg prednisolone daily

26
Q

What age group gets Kawasaki Disease

A

almost exclusively infants <5 years

27
Q

What is Kawasaki Disease

A

medium vessel vasculitis, that is a self-limiting acute febrile illness lasting around 5 days

28
Q

presentation of Kawasaki Disease

A 
fever 
polymorphic rash 
injected eyes 
mucosal erythema 
strawberry tongue
29
Q

Tx Kawasaki Disease

A

IVIG +/- aspirin

30
Q

What vessels does medium vessel vasculitis most commonly affect

A

skin
gut
kidneys

31
Q

What is Polyarteritis Nodosa

A

medium vessel vasculitis, with necrotising inflammatory lesions that affect arteries at vessel bifurcations resulting in microaneurysm formation in affected organs

32
Q

Shared features of small vessel vasculitis

A 
fever &amp; weight loss 
raised, non blanching purpuric rash 
arthralgia/arthritis 
mononeuritis multiplex 
glomerulonephritis 
lung opacities on xray
33
Q

What is Granulomatosis with Polyangiitis (GPA/Wegeners)

A

systemic vasculitis affecting small and medium sized vessels.
granulomatous inflammation involves the upper and lower respiratory tract, with necrotising glomerulonephritis

34
Q

Symptoms of GPA

A

ENT features -
nose bleeds, deafness, recurrent sinusitis, nasal crusting, “saddle nose” due to cartilage ischaemia

Respiratory symptoms -
cavitating lesions on xray

(also shared symptoms of small vessel vasculitis)

35
Q

What are ANCA

A

Anti-neutrophil cytoplasmic antibodies

A group of antibodies against antigens in the cytoplasm of neutrophil granulocytes

36
Q

ANCA pattern in GPA

A

cANCA

37
Q

What is Eosinophilic Granulomatosis with Polyangiitis (EGPA/Churg-Strauss)

A

systemic vasculitis affecting small and medium sized vessels.
eosinophilic and granulomatous inflammation involving the respiratory tract.
associated with asthma and eosinophilia

38
Q

ANCA pattern in EGPA

A

pANCA

39
Q

which small vessel vasculitis is most associated with PR3-ANCA

A

GPA

40
Q

small vessel vasculitis most associated with MPO-ANCA

A

MPA

41
Q

What is microscopic polyangiitis (MPA)

A

systemic vasculitis of small and medium vessels - not associated with granuloma formation.

42
Q

most important feature of MPA

A

glomerulonephritis

43
Q

Ix of small vessel vasculitis

A 
inflammatory markers 
FBC - anaemia of chronic disease
U&amp;E - renal involvement 
ANCA 
Urinalysis (renal vasculitis) 
CXR 
Biopsy of affected area
44
Q

Mx of ANCA-associated vasculitis

A

IV steroids and cyclophosphamide

45
Q

What is Henoch-Schonlein purpura?

A

Non-ANCA associated small vessel vasculitis

An acute IgA mediated disorder

46
Q

Most common organs affected by Henoch-Schonlein purpura?

A

smalls vessels of skin
GI tract
kidneys
joints

47
Q

Presentation of Henoch-Schonlein purpura?

A 
URTI precedes symptoms by a few weeks 
purpuric rash over buttocks and lower limbs 
abdo pain 
vomiting 
joint pain
48
Q

Tx of Henoch-Schonlein purpura?

A

self-limiting

symptoms tend to resolve over 8 weeks

49
Q

most common organism to cause Henoch-Schonlein purpura?

A

Group A Strep

50
Q

most common age group for HSP

A

children

51
Q

what disease is Polyarteritis Nodosa strongly associated with

A

Hepatitis B

Rheumatology (9 decks)

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