Vasculitis

Question 1

Vasculitis

Answer 1

Inflammation of blood vessels

Question 2

Vasculitis Pathogenesis

Answer 2

An inflammatory process causes transcription factor and collagen to be exposed.
Leads to the formation of a thrombosis.
The vessel wall becomes leaky.
Leading to aneurysm.
Fibrosis occurs due to fibrin deposition.
Thrombosis and fibrosis narrow the vessel.
Leading to Reduced Blood Flow.

Question 3

Vasculitis Vessel Changes

Answer 3

Vessel Wall thickening.
Vessel Stenosis
Occlusion of vessels with subsequent infarction.

Question 4

Large Vessel Vasculitis Types (2)

Answer 4

Giant cell arteritis

Takayasu Arteritis

Question 5

Large Vessel Vasculitis Early Features

Answer 5

Malaise
night Sweats
Weight loss 
Arthralgia
Fatigue
Claudicant Symptoms

Question 6

Large Vessel Vasculitis Late Features

Answer 6

Vascular Stenosis
Aneurysms
Reduced pulses and bruit.

Question 7

Large Vessel Vasculitis possible signs

Answer 7

Reduced pulses

Carotid Bruit

Question 8

Giant Cell Arteritis - Arteries affected (3)

Answer 8

Temporal Artery
Opthalmis Artery
Facial Arteries
- jaw claudication if in facial arteries

Question 9

Giant Cell Arteritis Epidemiology

Answer 9

Most common in women >50

Question 10

Giant Cell Arteritis Histology

Answer 10

Granulomas are present in the elastic lamina

Question 11

Takayasu Arteritis - Arteries affected

Answer 11

Affects the arteries off off the aortic arch

Question 12

Takayasu Arteritis Epidemiology

Answer 12

Most common in Asian Women <40

Question 13

Large Vessel Vasculitis Treatment

Answer 13

Treatment with 40-60mg Prednisolone

-Gradually reduced

Question 14

Medium Vessel Vasculitis Types (3)

Answer 14

Kawasaki Disease
Polyarteritis Nodosa
Buerger’s Disease

Question 15

Kawasaki Disease Features

Answer 15

Conjunctivitis
Rash over body
Adenopathy
Strawberry Tongue
Hands and feet swelling 
Fever

Question 16

Kawasaki Disease Treatment

Answer 16

Self-limiting.

Can be treated with IV Ig and Aspirin

Question 17

Polyarteritis Nodosa Pathology

Answer 17

Immune cells directly attack the endothelial cells.

Causes transmural necrosis. Leaves vessels prone to aneurysm.

Question 18

Polyarthritis Nodosa Investigations

Answer 18

Angiogram

-‘string of beads’

Question 19

Polyarthritis Nodosa Features

Answer 19

Can affect renal. lung and GI

• If renal, can lead to hypertension
• If GI, can lead to abdominal pain and GI bleeding

Question 20

Buerger’s Disease

Answer 20

Affecting vessels in fingers and toes

Question 21

Buerger’s Disease Risk Factor

Answer 21

Tobacco Use

Question 22

Buerger’s Disease Features

Answer 22

Blood clots occur.

Cause ulceration, auto-amputation and necrosis

Question 23

Small Vessel Vasculitis Types (4)

Answer 23

1. Granulomatosis with Polyangiitis (GPA)
2. Microscopic Polyangiitis (MPA)
3. Eosinophilic granulomatosis with polyangiitis (EGPA)
4. Henloch Schonlein Purpur

Question 24

Granulomatosis with Polyangiitis (GPA) Epidemiology

Answer 24

More common in men

Typical age 35-55

Question 25

Granulomatosis with Polyangiitis (GPA) Pathology

Answer 25

Granulomatous Inflammation of respiratory tract, small and medium vessels.

Question 26

Granulomatosis with Polyangiitis (GPA) Features

Answer 26

``` Affects nasopharynx, lungs and kidneys Sinusitis Bloody mucus Saddle Nose Deformities Difficulty Breathing Relapse is common ```

Question 27

Granulomatosis with Polyangiitis (GPA) Investigations

Answer 27

cANCA +ve | anti-PR3

Question 28

Granulomatosis with Polyangiitis (GPA) Treatment

Answer 28

ANCA +ve vasculitis treated with IV steroids and cyclophosphamide

Question 29

Microscopic Polyangiitis (MPA)

Answer 29

necrotising vasculitis with few immune deposits.

Question 30

Microscopic Polyangiitis (MPA) Features

Answer 30

Doesn't affect nasopharynx No granulomas present Can cause glomerulonephritis

Question 31

Microscopic Polyangiitis (MPA) Investigations

Answer 31

pANCA +ve | High eosinophil count.

Question 32

Eosinophilic granulomatosis with polyangiitis (EGPA)

Answer 32

Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels.

Question 33

Eosinophilic granulomatosis with polyangiitis (EGPA) Affected Sites

Answer 33

``` Sinuses Lungs GI Kidneys Skin nerve Heart ```

Question 34

Eosinophilic granulomatosis with polyangiitis (EGPA) Features

Answer 34

Granulomas are present | Associated with asthma

Question 35

Eosinophilic granulomatosis with polyangiitis (EGPA) Investigations

Answer 35

Anti-MPO anti-PR3 Increased eosinophils No ANCA

Question 36

Henloch Schonlein Purpura

Answer 36

Non-ANCA Vasculitis Acute IgA mediated disorder Often past history of URTI

Question 37

Henloch Schonlein Purpura Pathology

Answer 37

IgA targets endothelial cells via molecular mimicry

Question 38

Henloch Schonlein Purpur Features

Answer 38

palpable purpura due to fibrosis occurring in affected vessel - over buttocks and lower legs - abdominal pain - vomiting - joint pain Can occur in GI tract and kidneys GI- Haematuria Renal- IgA Nephropathy

Question 39

Henloch Schonlein Purpura Treatment

Answer 39

Can spontaneously resolve Self limiting Does not require treatment