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MSK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

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

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2
Q

What is primary vasculitis?

A

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

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3
Q

What is secondary vasculitis?

A

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

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4
Q

How is small vessel vasculitis divided?

A

into conditions asssociated with ANCA and those not

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5
Q

What are the common systemic symptoms in all vasculitides?

A

fever; malaise, wt loss, fatigue

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6
Q

What are two main causes of large vessel vasculitis?

A

Takavasu arteritis and Giant Cell arteritis

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7
Q

Who gets Takavaus arteritis?

A

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

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8
Q

what age gets giant cell arteritis?

A

over 50s

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9
Q

What are both large vessel vasculitides characterised by pathologically?

A

granulomatous infiltration of the walls and large vessels

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10
Q

What are the presenting features of the large vessel vasculitides?

A

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

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11
Q

What is temporal arteritis associated with?

A

PMR

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12
Q

What are the classic symptoms of GCA?

A

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

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13
Q

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

A

there are skip lesions

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14
Q

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

A

vessel wall thickening/stenodid/aneurysm or increased metabolic activity

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15
Q

What is the management of large vessel vasculitides?

A

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

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16
Q

What are the antibodies associated with the large vessel vasculitides?

A

there are none!

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17
Q

What is Kawasaki disease?

A

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

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18
Q

Who is Kawasakis seen in?

A

children under 5 years

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19
Q

What is polyarteritis nodosa?

A

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

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20
Q

What organs does Polyarteritis nodosa commonly affect?

A

skin; gut and kidneys

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21
Q

what disease is PAN often associated with ?

A

hepB

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22
Q

What was granulomatosis with polyangiitis called previusly?

A

Wegeners granulomatosis

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23
Q

What was eosinophilic granulomatosis with polyangiitis previosuly called?

A

Churg Strauss

24
Q

What is GPA?

A

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

25
Q

What is churg strauss?

A

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

26
Q

What is microscopic polyangiitis?

A

necrotising vasculitis with few immune deposits. necrotising glomerulnephritis very common

27
Q

Who is GPA seen in?

A

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

28
Q

What are the 4 features of GPA?

A

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

29
Q

What are the ENT features of GPA?

A

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

30
Q

What are the resp features of GPA?

A

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

31
Q

What are the cutaneous features of GPA?

A

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

32
Q

What are the renal feautres of GPA?

A

necrotiing glomerulonephritis

33
Q

What does necrotising glomerulonephritis manifest as?

A

blood and protein in the urine

34
Q

How is the nervous system affected in GPA?

A

mononeuritis multiplex; sensorimotor polyneuropathy; cranial nerve palsy

35
Q

What happens in mononeuritis multiplex?

A

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

36
Q

What are the ocular features of GPA?

A

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

37
Q

What is the main difference between EGPA nad GPA?

A

the presence of late onset asthma and a high eosinophil count

38
Q

What is seen on hisotlogy of EGPA?

A

proof of vasculitis; granulomas with extravascular eosinophils

39
Q

What else is different in EGPA compared to GPA?

A

ENT involvement is much rarer in EGPA

40
Q

What are the ANCA antibodies?

A

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

41
Q

What ANCA is associated iwth GPA?

A

cANCA

42
Q

What ANCA are MPO and EGPA associated with?

A

pANCA

43
Q

What other antibody is associated with GPA?

A

anti-PR3

44
Q

What antibody is associated with EGPA and MPA

A

anti-MPO

45
Q

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

A

they are more specific

46
Q

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

A

levels vary with disease actviity

47
Q

What is the difference between generalised and systemmic AAV?

A

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

48
Q

How is localised/early systemic AAV treated?

A

methotrexate and steroids

49
Q

How is generalised/ systemic AAV treated?

A

cyclophosphamide and steroid

rituximab and steroids then followed by azathioprine for maintenance

50
Q

How is refractory AAV treated?

A

IV immunoglobulins

rituximab

51
Q

What is Henoch-Schonlein Purpura?

A

an acute immunoglobulin IgA mediated disrorder of the small vessels

52
Q

Who gets Henoch-Schonlein Purpura?

A

children aged 2-11

53
Q

What usually precedes HSP?

A

an infeection- URTI; pharngeal or GI infections

54
Q

What is the most common pathogen preceding HSP?

A

group A strep

55
Q

When would the preceding illness before HSP be?

A

1-3 weeks

56
Q

How does HSP present?

A

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

57
Q

What is the prognosis for HSP?

A

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

MSK (39 decks)

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