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Flashcards in Vasculitis Deck (57):
1

What is vasculitis?

inflammation of blood vessels, often with ischaemia, necrosis and organ inflam

2

What is primary vasculitis?

results from an inflammatory response that targets the vessel walla dn has no known cause- sometimes autoimmune

3

What is secondary vasculitis?

may be triggered by an infection, a drug or a toxin or may occur as part of another inflam disorder or cancer

4

How is small vessel vasculitis divided?

into conditions asssociated with ANCA and those not

5

What are the common systemic symptoms in all vasculitides?

fever; malaise, wt loss, fatigue

6

What are two main causes of large vessel vasculitis?

Takavasu arteritis and Giant Cell arteritis

7

Who gets Takavaus arteritis?

the under 40s; more common in females and in asian (esp. Japanese) populations

8

what age gets giant cell arteritis?

over 50s

9

What are both large vessel vasculitides characterised by pathologically?

granulomatous infiltration of the walls and large vessels

10

What are the presenting features of the large vessel vasculitides?

bruit- most commonly the carotid; BP difference of extremities; claudication; carotodynia or vessel tenderness; HT

11

What is temporal arteritis associated with?

PMR

12

What are the classic symptoms of GCA?

unilateral temporal headache; scalp tenderness and jaw claudication; prominent temporal arteries with reduced pulsation; vision impairmnet

13

What do you have to be careful with taking biopies in the vasculitides?

there are skip lesions

14

Why might an MR angiogram or PET CT be done in the large vessel vasculitides?

vessel wall thickening/stenodid/aneurysm or increased metabolic activity

15

What is the management of large vessel vasculitides?

40-60mg prednisolone (60mg if visual disturbance); grad reduced over 18-24 months.

16

What are the antibodies associated with the large vessel vasculitides?

there are none!

17

What is Kawasaki disease?

medium vessel vasculitis which can occur in the coronary arteries where aneurysms can develop

18

Who is Kawasakis seen in?

children under 5 years

19

What is polyarteritis nodosa?

necrotising inflam lesions that affect medium arteries at vesssel bifurcations resulting in microanurysm fommration and aneurysms

20

What organs does Polyarteritis nodosa commonly affect?

skin; gut and kidneys

21

what disease is PAN often associated with ?

hepB

22

What was granulomatosis with polyangiitis called previusly?

Wegeners granulomatosis

23

What was eosinophilic granulomatosis with polyangiitis previosuly called?

Churg Strauss

24

What is GPA?

granulomatous inflam of the resp tract, small and med vessels. necrotising glowerulonephritis common

25

What is churg strauss?

eosinophilic granulomatous inflam of the resp tract, small and med vessels and associated with astham

26

What is microscopic polyangiitis?

necrotising vasculitis with few immune deposits. necrotising glomerulnephritis very common

27

Who is GPA seen in?

individuals of northern European descent; male-to-female of 1.5:1; 35-55yo

28

What are the 4 features of GPA?

nasal or oral inflam; abnormal chest radiograph; urinary sediment; granulomatous inflam on biopsy

29

What are the ENT features of GPA?

sinusitis; nasal crusting; epistaxis; mouth ulcers; deafness; "saddle nose" due to cartilage ischaemia

30

What are the resp features of GPA?

pulmonary infiltrates; cough; haemoptysis; diffuse alveolar haemorrhage; cavitating nodules on CXR

31

What are the cutaneous features of GPA?

palpable purpura (non-blanching)- worse in gravity areas- feet, lower limbs; cutaneous ulcers

32

What are the renal feautres of GPA?

necrotiing glomerulonephritis

33

What does necrotising glomerulonephritis manifest as?

blood and protein in the urine

34

How is the nervous system affected in GPA?

mononeuritis multiplex; sensorimotor polyneuropathy; cranial nerve palsy

35

What happens in mononeuritis multiplex?

peripheral nerves are picked off by inflam in blood vessels eg foot and wrist drop

36

What are the ocular features of GPA?

conjuctivities; episcleritis; uveitis; optic nerve vasculitis; retinal artery occlusion; proptosis

37

What is the main difference between EGPA nad GPA?

the presence of late onset asthma and a high eosinophil count

38

What is seen on hisotlogy of EGPA?

proof of vasculitis; granulomas with extravascular eosinophils

39

What else is different in EGPA compared to GPA?

ENT involvement is much rarer in EGPA

40

What are the ANCA antibodies?

a group of autoantibodies against antigens in the cytoplasm of neutrophil granulocytes

41

What ANCA is associated iwth GPA?

cANCA

42

What ANCA are MPO and EGPA associated with?

pANCA

43

What other antibody is associated with GPA?

anti-PR3

44

What antibody is associated with EGPA and MPA

anti-MPO

45

why are the antibodies for GPA better than those for EGPA and MPA?

they are more specific

46

Why are ANCA, anti-PR3 and anti-MPO antibodies useful in monitoring disease?

levels vary with disease actviity

47

What is the difference between generalised and systemmic AAV?

generlaised is when organs are threatened whereas in systemic vital organs are failing

48

How is localised/early systemic AAV treated?

methotrexate and steroids

49

How is generalised/ systemic AAV treated?

cyclophosphamide and steroid
rituximab and steroids then followed by azathioprine for maintenance

50

How is refractory AAV treated?

IV immunoglobulins
rituximab

51

What is Henoch-Schonlein Purpura?

an acute immunoglobulin IgA mediated disrorder of the small vessels

52

Who gets Henoch-Schonlein Purpura?

children aged 2-11

53

What usually precedes HSP?

an infeection- URTI; pharngeal or GI infections

54

What is the most common pathogen preceding HSP?

group A strep

55

When would the preceding illness before HSP be?

1-3 weeks

56

How does HSP present?

pupuric rash over the buttocks and lower limbs; colicky abdo pain; bloody diarrhoea; joint pain (and swelling) renal involvement

57

What is the prognosis for HSP?

ususally self-limiting, tend to resolve within 8 weeks, although relapses may occur