Vasculitis

Question 1

What is the most common form of systemic vasculitis in adults?

Answer 1

giant cell arteritis

Question 2

what histologically is seen in giant cell arteritis?

Answer 2

transmural inflammation (involving intima, media and adventita) as well as patchy granulomatous infiltration of lymphocytes, macrophages and multinucleated giant cells

Question 3

why can giant cell arteritis cause distal ischaemia?

Answer 3

the artery wall becomes thickened due to the inflammation. This causes luminal narrowing.

Question 4

what should you always consider in a patient over 50 y/o with a new onset headache and raised CRP/PV/ESR?

Answer 4

giant cell arteritis

Question 5

what artery is commonly affected in giant cell arteritis?

Answer 5

temporal artery

Question 6

what is the most serious consequence of giant cell arteritis in the temporal artery?

Answer 6

permanent visual impairement

Question 7

describe the headache commonly seen in patients with temporal arteritis?

Answer 7

continuous and located in the temporal or occipital areas

focal tenderness on direct palpation

Question 8

why can patient with temporal arteritis experience jaw claudication?

Answer 8

ischamia of the maxillary artery

Question 9

what is jaw claudication?

Answer 9

jaw fatigue or discomfort during chewing or prolonged speaking

Question 10

what is the most definitive test for suspected giant cell arteritis?

Answer 10

temporal artery biopsy

Question 11

compare specificity to sensitivity in terms of a temporal artery biopsy for GCA?

Answer 11

high specificity

low sensitivity

Question 12

why does temporal artery biopsy have a low sensitivity for GCA?

Answer 12

because there is patchy involvement- some segments of temporal artery might be normal

Question 13

what is the treatment for temporal arteritis with visual symptoms?

Answer 13

60mg prednisolone tapered over 2 years

Question 14

what is the treatment for temoral arteritis without visual symptoms?

Answer 14

40mg prednisolone tapered over 2 years

Question 15

when should treatment with steroids be started for a patient with suspected temporal arteritis?

Answer 15

as soon as diagnosis is suspected

don’t wait for biopsy results

Question 16

what is large vessel vasculitis?

Answer 16

primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its branches

Question 17

what are the 2 major categories of large vessel vasculitis?

Answer 17

Takayasu arteritis

Giant cell arteritis

Question 18

who tends to get takayasu?

Answer 18

young women

mainly in east asian countries

Question 19

what are the clinical signs of large vessel vasculitis?

Answer 19

reduced pulses

bruits

Question 20

what is the mainstay treatment of large vessel vasculitis?

Answer 20

40-60mg prednisolone
tapering doses

steroid sparing agents eg methotrexate and azathioprine may be used

Question 21

what are the 2 main classes of small vessel vasculitis?

Answer 21

ANCA-associated

Non-ANCA- associated

Question 22

within ANCA-associated small vessel vasculitis, what is the further subdivision?

Answer 22

granulomas - GPA or EGPA

no granulomas- MPA

Question 23

What are the main differences between EGPA and GPA?

Answer 23

EGPA- eosinophilia, late onset asthma, cANCA

GPA- common ENT symptoms, pANCA

Question 24

what is the most important complication of microscopic polyangitis?

Answer 24

glomerulonephritis

Question 25

what is the management of ANCA-associated vasculitis?

Answer 25

IV steroids | cyclophosphamide

Question 26

what is Henoch-Schonlein purpura?

Answer 26

an acute IgA mediated disorder which commonly affects children after a history of a upper respiratory tract infection

Question 27

what is the treatment of Henoch schonein purpura?

Answer 27

self-limiting

Question 28

what is vasculitis?

Answer 28

inflammation of blood vessels, often with ischamia, necrosis and organ inflammation

Question 29

why can vasculitis lead to blood clot formation?

Answer 29

lumen becomes narrower and irregular which promotes blood clots formting

Question 30

what are the 2 main classes of medium vessel vaculitis-?

Answer 30

polyarteritis nodosa | kawasaki disease

Question 31

what are both takayasu arteritis and giant cell arteritis characterised by?

Answer 31

granulomatous infiltration of the walls of large vessels

Question 32

why are granulomas generally formed in the body?

Answer 32

to wall off a 'threat' from the body | one that can't be expelled

Question 33

what type of cells from a granuloma merge together to form giant cells?

Answer 33

macrophages | giant cell arteritis

Question 34

what are the main clinical findings of large vessel vasculitis?

Answer 34

- bruit (usually carotid) - blood pressure difference of extremities - vessel tenderness (usually unilateral) - hypertension

Question 35

what clinical sign may indicate temporal arteritis?

Answer 35

prominent temporal arteries | reduced pulsation

Question 36

who tends to get kawasaki disease?

Answer 36

children under 5

Question 37

aneurysms to what arteries are a significant risk in kawaski disease?

Answer 37

coronary arteries

Question 38

when do you choose to give treatment in kawaski disease?

Answer 38

when coronary arteries are affected | if they aren't affected this condition will subside without treatment

Question 39

where in the blood does polyarteritis nodosa tend to affect?

Answer 39

birfucations

Question 40

what can result from the necrotising inflammatory lesions caused by polyarteritis nodosa?

Answer 40

microaneurysm and aneurysm formation

Question 41

what viral disease is polyarteritis nodosa associated with?

Answer 41

hepatitis B

Question 42

what is the new name of Wegner's granulomatosis?

Answer 42

granulomatosis with polyangiitis (GPA)

Question 43

what is the new name of Churg-Strauss syndrome?

Answer 43

Eosinophilic granulomatosis with polyangitiis (EGPA)

Question 44

what respiratory disease to patients with eosinophilc granulomatosis with polyangitiis frequently get?

Answer 44

late-onset asthma

Question 45

what is a 'saddle nose'?

Answer 45

collapse of nasal cartilage

Question 46

what causes a saddle nose?

Answer 46

collapse of nasal cartilage caused by cartilage ischaemia | ENT features such as this present in GPA

Question 47

what are the main 2 cutaneous features of small vessel vasculitis?

Answer 47

``` ulcers palpable purpura (ie raised purpura) ```

Question 48

why must you always do a urinalysis in a patient with suspected small vessel vasculitis?

Answer 48

glomerulonephritis can occur this is asymptomatic until late stage so you actively try to catch it early

Question 49

what is mononeuritis multiplex?

Answer 49

impairment of blood supply to nerves (usually long nerves eg perineal or radial)

Question 50

what is the clinical feature when a patient with mononeuritis multiplex of the common perineal nerve can't extend their ankle?

Answer 50

drop foot

Question 51

what are ANCAs?

Answer 51

anti-neutrophil cytoplasmic antibodies: | autoantibodies against antigens in the cytoplasm of neutrophil granulocytes

Question 52

what type of ANCAs do ANCA-associated small vessel vasculitis have?

Answer 52

GPA and EGPA- cANCA MPA- pANCA

Question 53

what is localised ANCA-associated vasculitis?

Answer 53

upper/lower resp tract disease | no other systemic involvement or constitutional symptoms

Question 54

what is early systemic ANCA-associated vasculitis?

Answer 54

any involvement without organ or life threatening involvement

Question 55

what is generalised ANCA-associated vasculitis?

Answer 55

renal or other organ threatening involvement

Question 56

what is systemic ANCA-associated vaculitis?

Answer 56

renal or other vital organ failure

Question 57

what is refractory ANCA-associated vasculitis?

Answer 57

progressive disease unreponsive to steroids + cyclophosphamide

Question 58

what is the treatment of localised ANCA-associated vasculitis?

Answer 58

methotrexate + steroids

Question 59

what is the treatment of early systemic ANCA- associated vasculitis?

Answer 59

methotrexate + steroids

Question 60

what is the treament of generalised ANCA- associated vasculitis?

Answer 60

cyclophosphamide + steroids | rituximab + steroids alternative

Question 61

what is the treatment of systemic ANCA- associated vasculitis?

Answer 61

cyclophosphamide + steroids | rituximab + steroids alternative

Question 62

what is the treatment of refractory ANCA-associated vasculitis?

Answer 62

IV immunoglobulins | rituximab

Question 63

what is Henoch-Schonlein Purpura?

Answer 63

a non-ANCA associated small vessel vasculitis acute IgA mediated disorder

Question 64

who tends to get Henoch-Schonlein Purpura?

Answer 64

children 2- 11 | but rarely infants

Question 65

what usually precedes Henoch-Schonlein Purpura?

Answer 65

an upper respiratory infection, pharyngeal infection, GI infection (a few weeks before)

Question 66

what is the most common organism involved in the infection preceding Henoch-Schonlein Purpura?

Answer 66

Group A Strep

Question 67

what is the treatment of Henoch-Schonlein Purpura?

Answer 67

usually self limiting if evidence of gut or renal involvement- hospital admittance with supportive therapy

Question 68

what is the difference between purpura and telangiectasia?

Answer 68

purpura doesn't blanche | telangiectasia does