Vasculitis

Question 1

What is vasculitis?

Answer 1

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

Question 2

What is primary vasculitis?

Answer 2

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

Question 3

What is secondary vasculitis?

Answer 3

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

Question 4

How is small vessel vasculitis divided?

Answer 4

into conditions asssociated with ANCA and those not

Question 5

What are the common systemic symptoms in all vasculitides?

Answer 5

fever; malaise, wt loss, fatigue

Question 6

What are two main causes of large vessel vasculitis?

Answer 6

Takavasu arteritis and Giant Cell arteritis

Question 7

Who gets Takavaus arteritis?

Answer 7

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

Question 8

what age gets giant cell arteritis?

Answer 8

over 50s

Question 9

What are both large vessel vasculitides characterised by pathologically?

Answer 9

granulomatous infiltration of the walls and large vessels

Question 10

What are the presenting features of the large vessel vasculitides?

Answer 10

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

Question 11

What is temporal arteritis associated with?

Answer 11

PMR

Question 12

What are the classic symptoms of GCA?

Answer 12

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

Question 13

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

Answer 13

there are skip lesions

Question 14

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

Answer 14

vessel wall thickening/stenodid/aneurysm or increased metabolic activity

Question 15

What is the management of large vessel vasculitides?

Answer 15

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

Question 16

What are the antibodies associated with the large vessel vasculitides?

Answer 16

there are none!

Question 17

What is Kawasaki disease?

Answer 17

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

Question 18

Who is Kawasakis seen in?

Answer 18

children under 5 years

Question 19

What is polyarteritis nodosa?

Answer 19

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

Question 20

What organs does Polyarteritis nodosa commonly affect?

Answer 20

skin; gut and kidneys

Question 21

what disease is PAN often associated with ?

Answer 21

hepB

Question 22

What was granulomatosis with polyangiitis called previusly?

Answer 22

Wegeners granulomatosis

Question 23

What was eosinophilic granulomatosis with polyangiitis previosuly called?

Answer 23

Churg Strauss

Question 24

What is GPA?

Answer 24

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

Question 25

What is churg strauss?

Answer 25

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

Question 26

What is microscopic polyangiitis?

Answer 26

necrotising vasculitis with few immune deposits. necrotising glomerulnephritis very common

Question 27

Who is GPA seen in?

Answer 27

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

Question 28

What are the 4 features of GPA?

Answer 28

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

Question 29

What are the ENT features of GPA?

Answer 29

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

Question 30

What are the resp features of GPA?

Answer 30

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

Question 31

What are the cutaneous features of GPA?

Answer 31

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

Question 32

What are the renal feautres of GPA?

Answer 32

necrotiing glomerulonephritis

Question 33

What does necrotising glomerulonephritis manifest as?

Answer 33

blood and protein in the urine

Question 34

How is the nervous system affected in GPA?

Answer 34

mononeuritis multiplex; sensorimotor polyneuropathy; cranial nerve palsy

Question 35

What happens in mononeuritis multiplex?

Answer 35

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

Question 36

What are the ocular features of GPA?

Answer 36

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

Question 37

What is the main difference between EGPA nad GPA?

Answer 37

the presence of late onset asthma and a high eosinophil count

Question 38

What is seen on hisotlogy of EGPA?

Answer 38

proof of vasculitis; granulomas with extravascular eosinophils

Question 39

What else is different in EGPA compared to GPA?

Answer 39

ENT involvement is much rarer in EGPA

Question 40

What are the ANCA antibodies?

Answer 40

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

Question 41

What ANCA is associated iwth GPA?

Answer 41

cANCA

Question 42

What ANCA are MPO and EGPA associated with?

Answer 42

pANCA

Question 43

What other antibody is associated with GPA?

Answer 43

anti-PR3

Question 44

What antibody is associated with EGPA and MPA

Answer 44

anti-MPO

Question 45

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

Answer 45

they are more specific

Question 46

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

Answer 46

levels vary with disease actviity

Question 47

What is the difference between generalised and systemmic AAV?

Answer 47

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

Question 48

How is localised/early systemic AAV treated?

Answer 48

methotrexate and steroids

Question 49

How is generalised/ systemic AAV treated?

Answer 49

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

Question 50

How is refractory AAV treated?

Answer 50

IV immunoglobulins | rituximab

Question 51

What is Henoch-Schonlein Purpura?

Answer 51

an acute immunoglobulin IgA mediated disrorder of the small vessels

Question 52

Who gets Henoch-Schonlein Purpura?

Answer 52

children aged 2-11

Question 53

What usually precedes HSP?

Answer 53

an infeection- URTI; pharngeal or GI infections

Question 54

What is the most common pathogen preceding HSP?

Answer 54

group A strep

Question 55

When would the preceding illness before HSP be?

Answer 55

1-3 weeks

Question 56

How does HSP present?

Answer 56

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

Question 57

What is the prognosis for HSP?

Answer 57

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