Vasculitis

Question 1

What is vasculitis

Answer 1

inflammation of blood vessels

Question 2

generally, how is vasculitis classified

Answer 2

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

Question 3

2 types of large vessel vasculitis

Answer 3

Giant Cell Arteritis

Takayasu arteritis

Question 4

2 types of medium vessel vasculitis

Answer 4

Kawasaki disease

Polyarteritis nodosa

Question 5

Types of small vessel vasculitis

Answer 5

ANCA-associated

Non- ANCA associated

Question 6

Types of ANCA-associated small vessel vasculitis

Answer 6

Microscopic polyangitis

Granulomatosis with polyangitis (Wegener)

Eosinophilic granulomatosis with polyangitis (Churg-Strauss)

Question 7

Types of non-ANCA associated small vessel vasculitis

Answer 7

Cryoglobulinemic vasculitis

IgA vasculitis (Henoch-Schonlein)

Question 8

what vessels do Large vessel arteritis affect

Answer 8

aorta and its major branches

Question 9

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

Answer 9

Age of onset

GCA occurs rarely before 50 years

TA occurs rarely after 50 years

Question 10

Describe pathology of GCA

Answer 10

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.

Question 11

condition commonly associated with GCA

Answer 11

polymyalgia rheumatica

Question 12

artery most commonly affected by GCA

Answer 12

temporal artery

Question 13

presentation of GCA

Answer 13

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

constitutional symptoms:
fatigue
malaise
fever

Question 14

what symptom of GCA is the most significant causes of morbidity

Answer 14

visual disturbances

Question 15

what causes the jaw claudication seen in GCA

Answer 15

ischaemia of the maxillary artery

Question 16

visual disturbances seen in GCA

Answer 16

unilateral visual blurring
unilateral visial loss
painless
diplopia

Question 17

definitive test for GCA

Answer 17

temporal artery biopsy

Question 18

main problem with temporal artery biopsy as diagnostic test for GCA

Answer 18

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

Question 19

typical biopsy findings in GCA

Answer 19

mononuclear infiltration or granulomatous inflammation with multinucleated giant cells

Question 20

Mx of GCA

Answer 20

40mg pred if no visual symptoms

60mg pred if visual symptoms

Question 21

when should treatment of GCA be started

Answer 21

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

Question 22

inflammatory markers in GCA

Answer 22

plasma viscosity
ESR
CRP
- all elevated

Question 23

most common group of people to get Takayasu Arteritis

Answer 23

females <40 years and Asian populations

Question 24

Consequence of untreated large vessel vasculitis

Answer 24

vascular stenosis and aneurysms

- results in reduced pulses and (carotid) bruits

Question 25

Tx Takayasu Arteritis

Answer 25

same as for GCA | 40-60mg prednisolone daily

Question 26

What age group gets Kawasaki Disease

Answer 26

almost exclusively infants <5 years

Question 27

What is Kawasaki Disease

Answer 27

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

Question 28

presentation of Kawasaki Disease

Answer 28

``` fever polymorphic rash injected eyes mucosal erythema strawberry tongue ```

Question 29

Tx Kawasaki Disease

Answer 29

IVIG +/- aspirin

Question 30

What vessels does medium vessel vasculitis most commonly affect

Answer 30

skin gut kidneys

Question 31

What is Polyarteritis Nodosa

Answer 31

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

Question 32

Shared features of small vessel vasculitis

Answer 32

``` fever & weight loss raised, non blanching purpuric rash arthralgia/arthritis mononeuritis multiplex glomerulonephritis lung opacities on xray ```

Question 33

What is Granulomatosis with Polyangiitis (GPA/Wegeners)

Answer 33

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

Question 34

Symptoms of GPA

Answer 34

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)

Question 35

What are ANCA

Answer 35

Anti-neutrophil cytoplasmic antibodies A group of antibodies against antigens in the cytoplasm of neutrophil granulocytes

Question 36

ANCA pattern in GPA

Answer 36

cANCA

Question 37

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

Answer 37

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

Question 38

ANCA pattern in EGPA

Answer 38

pANCA

Question 39

which small vessel vasculitis is most associated with PR3-ANCA

Answer 39

GPA

Question 40

small vessel vasculitis most associated with MPO-ANCA

Answer 40

MPA

Question 41

What is microscopic polyangiitis (MPA)

Answer 41

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

Question 42

most important feature of MPA

Answer 42

glomerulonephritis

Question 43

Ix of small vessel vasculitis

Answer 43

``` inflammatory markers FBC - anaemia of chronic disease U&E - renal involvement ANCA Urinalysis (renal vasculitis) CXR Biopsy of affected area ```

Question 44

Mx of ANCA-associated vasculitis

Answer 44

IV steroids and cyclophosphamide

Question 45

What is Henoch-Schonlein purpura?

Answer 45

Non-ANCA associated small vessel vasculitis | An acute IgA mediated disorder

Question 46

Most common organs affected by Henoch-Schonlein purpura?

Answer 46

smalls vessels of skin GI tract kidneys joints

Question 47

Presentation of Henoch-Schonlein purpura?

Answer 47

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

Question 48

Tx of Henoch-Schonlein purpura?

Answer 48

self-limiting | symptoms tend to resolve over 8 weeks

Question 49

most common organism to cause Henoch-Schonlein purpura?

Answer 49

Group A Strep

Question 50

most common age group for HSP

Answer 50

children

Question 51

what disease is Polyarteritis Nodosa strongly associated with

Answer 51

Hepatitis B