Vasculitis

Question 1

What is vasculitis?

Answer 1

Inflammation of blood vessels, often with ishemia, necrosis and organ inflammation

Question 2

What blood vessels can vasculitis affect?

Answer 2

Arteries, arterioles, vein, venules or capillaties

Question 3

What is primary vasculitis?

Answer 3

Results from an inflammatory response that targets vessel walls with no known cause. Commonly autoimmune

Question 4

What is secondary vasculitis?

Answer 4

Triggered by an infection, drug (e.g. cocaine) or a toxin that may occur as part of another inflammatory disorder or cancer

Question 5

What will occur at a cellualr level in vasculitis?

Answer 5

Release of inflammatory cytokines activating T cells and vascular inflammation. This promotes granuloma formation and macrophage activation.
This will lead to inflammation, endothelial damage, disruption of elastic lamina and intimal hyperperplasia

Question 6

What are examples of large-vessel vsaculitis?

Answer 6

Takayasu arteritiis

Giant Cell arteritis

Question 7

What are examples of medium-vessel vasculitis?

Answer 7

Polyarteritis nodosa

Kawasaki disease

Question 8

What are ANCA-positive small vessel vsaculitis?

Answer 8

Microscopic polyangitis
Granulomatosis with polyangitis (wegner’s)
Esoinophilic granulomatosis with polyangitis (churg-strauss)

Question 9

What will churg strauss also present with along with vasculitis?

Answer 9

Rhinorea, asthma and eosinophilia

Question 10

What non-ANCA small vessel vasculitis is Ig-A dominant?

Answer 10

Henoch-Sschnelin purpura

Question 11

What are non-ANCA, immune complex small vessel vasculitis?

Answer 11

Cryoglubinemic vasculitis
IgA vasculitis (henoch-Shconlein)

Question 12

What non-ANCA small vessel vasculitis has high serum cryoglobulin?

Answer 12

Cryglobulinemia

Question 13

What systemic symptoms can present with vasculitis?

Answer 13

Fever
Malaise
Weight loss
Fatigue

Question 14

Who will TA affect?

Answer 14

Under 40
Females
Asian population

Question 15

Who wil GCA affect?

Answer 15

Over 50

Question 16

What are the presenting features of large cell vasculitis?

Answer 16

Bruit - most commonly around carotid artery
BP difference in extremities
Claudication

Question 17

What muscle disease does GCA have an association with?

Answer 17

Polymyalgia Rheumatica

Question 18

What are the symptoms of GCA?

Answer 18

Unilateral temporal headache
Scalp tenderness
Jaw claudication

Question 19

What occular symptom can GCA present with?

Answer 19

Blindness due to ischaemia of optic nerve and opthalmic artery

Question 20

What are the investigations for GCA?

Answer 20

ESR, PV and CRP
Temporal artery biopsy
MR anigiogram or PET CT

Question 21

How is GCA managed?

Answer 21

40-60mg prednisolone

Higher dose if visual loss

Question 22

What is the pathology of GPA?

Answer 22

Granulomatous inflammation of resp tract, small and medium vessels
Necrotising glomerulonephritis

Question 23

What is the pathology of EGPA?

Answer 23

Eosinophilic granulomatous inflammation of resp tract, small and medium vessels
Associated with asthma

Question 24

What is the pathology of MPA?

Answer 24

Necrotising vasculitis with few immune deposits

Necrotising glomerulonephritis very common

Question 25

Who is likey to get GPA?

Answer 25

Slightly more common in males 35-55 years Constitutional symptoms and arthralgia are common

Question 26

What is the classification of GPA?

Answer 26

Nasal or oral inflammation Abnormal chest radiograph - nodules, fixed infiltrates or cavities Urinary sediment - microhaematuria or red cell casts Granulomatous inflammation on biopsy

Question 27

What ENT features can GPA show?

Answer 27

``` Sinusitis Nasal crusting Epistaxis Mouth ulcers Saddle nose ```

Question 28

What resp features can GPA show?

Answer 28

``` Pulmonary infiltrates Cough Heamoptysis Diffuse alveolar haemorrhage CAvitating nodules ```

Question 29

What cutaneous features can GPA show?

Answer 29

Palpable purpura | Cutaneous ulcers

Question 30

What renal features can GPA show?

Answer 30

Necrotising golemrulonephritis

Question 31

What nervous system features can GPA show?

Answer 31

Mononeuritis multiplex Sensorimotor polyneuropathy Cranial nerve palsies

Question 32

What occular features can GPA show?

Answer 32

``` Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusion ```

Question 33

What is the main difference between GPA and EGPA?

Answer 33

EGPA will occur with late onset asthma and a high eosinophil count

Question 34

What is the critera for EGPA?

Answer 34

Ashtma Eosinophilia of more than 10% in peripheral blood Paranasal sinusitis Pulmonary infiltrates Histological proof of vasculitis with extravascular eosinophils Mononeuritis multiplex or polyneuropathy

Question 35

What condition will have c-ANCA and PR3 antibodies?

Answer 35

GPA

Question 36

What condition will have p-ANCA MPO antibodies?

Answer 36

MPA and EGPA

Question 37

How is anca associated vasculitis managed?

Answer 37

Cyclophosphamide

Question 38

What is henoch-schonlein purpura (HSP)?

Answer 38

Acute IgA mediated disorder that causes generalised vasculitis

Question 39

What can HSP affect?

Answer 39

``` Small vessels of skin GI trct Kidneys Joints RARE: lungs and CNS ```

Question 40

Who is likely to get HSP?

Answer 40

Children aged 2-11

Question 41

What will usually precede HSP?

Answer 41

Preceding URTI, pharyngeal infection or GI infection | Commonly group A streptococci

Question 42

How will HSP present?

Answer 42

``` Purpuric rash over buttocks and lower limbs Colickly abdo pain Bloody diarrhoea Joint pain +/- swelling Renal involvement in 50% ```

Question 43

How is HSP managed?

Answer 43

Usually self-limiting Symptoms tend ot reslove within 8 weeis Essential to perform urinalysis to screen for renal involvement