Vasculitis

Question 1

What occurs in large vessel vasculitis?

Answer 1

Chronic granulomatous inflammation usually of the aorta and main branches.

Question 2

Two main types of large vessel vasculitis

Answer 2

Takayasu under 50 years

Giant Cell arteritis over 50 years

Question 3

Takayasu

Answer 3

Under 50 year old East Asian female.
Claudication and bruits usually in the carotids.
Blood pressure differences in extremities

Question 4

Giant Cell Arteritis

Answer 4

Over 50

Temporal artery involvement is common but can also affect other large arteries.

Question 5

If untreated what can occur in large vessel vasculitis.

Answer 5

Vascular stenosis and distal ischaemia and aneurysm.

Question 6

What investigations are used in large vessel vasculitis?

Answer 6

FBC- raised CRP and plasma viscosity
MR angiography- shows stenosis and thickened walls
Temporal artery biopsy - GCA
PET scan- shows increased metabolic activity in large vessels.

Question 7

Treatments for large vessel vasculitis?

Answer 7

40-60mg of prednisolone.

Steroid sparing methotrexate may be used.

Question 8

What is the primary medium vessel vasculitis and who does it usually affect?

Answer 8

Kawasaki

Usually children under 5

Question 9

What are the to main types of small vessel vasculitis?

Answer 9

ANCA +ve

ANCA -ve

Question 10

How are all small vessel vasculitis presentations similar?

Answer 10

Fever and weight loss
Raised non blanching purpuric rash
Arthralgia
Glomerulonephritis
Lung opacities on x-ray

Question 11

Name 4 types of ANCA positive vasculitis

Answer 11

Granulomatosis with polyangitis
Microscopic Polyangitis
Renal Limited vasculitis
Eosinophilc granulomatosis with polyangitis

Question 12

What is Granulomatosis with polyangitis also known as?

Answer 12

Wegeners

Question 13

How does GPA usually present?

Answer 13

Sinusitis
Mouth Ulcers
Saddle Nose
Otitis media
Deafness
Uveitis and conjunctivitis

Question 14

GPA with respiratory involvement

Answer 14

Haemoptysis

Pulmonary infiltrates and cavitating nodules on X-ray

Question 15

GPA with cutaneous involvement

Answer 15

palpable purpura

and ulceration

Question 16

GPA with renal involvement

Answer 16

Necrotising glomerulonephritis

Question 17

GPA with CNS involvement

Answer 17

Nerve palsy

Question 18

What is GPA associated with?

Answer 18

cANCA and PR3 proteinase 3

Question 19

Is GPA more common in men or women?

Answer 19

M:F 1.5:1
Over 35
90% N.Europe

Question 20

How is eosiniphilc granulmatoses with polyangitis different to GPA

Answer 20

Presents the same but Late onset Asthma
Raised blood eosinophil count
Rhinitits

Question 21

What is different immunological between EGPA and GPA

Answer 21

EGPA is pANCA
GPA in cANCA
Low complement levels in both

Question 22

What antibodies are associated with EGPA

Answer 22

pANCA

Anti NPO

Question 23

Small Vessel Vasculitis Investigations

Answer 23

FBC- PV and CRP raised
Anaemie- common in chronic
Immunology- ANCA Biopsy
Imaging- Chest Xray

Question 24

Investigations if suspected renal involvement?

Answer 24

Urine dipstick for proteinuria

Urgent kidney biopsy

Question 25

Management of ANCA +ve disease

Answer 25

``` Localised/Early - Methotrexate Systemic/Agresive/Late - Cyclophosphamide and steroid - Rituximab and steroid Followed by azathioprine or methotrexate once controlled. ```

Question 26

What is used in refractory ANCA +ve disease?

Answer 26

IV immunoglobulins and rituximab

Question 27

Henoch Schnlien Purpura

Answer 27

ANCA -ve disease | Ig-A dominant immune deposits.

Question 28

Who is usually affected by HSP?

Answer 28

75% children with a preceding infection Upper respiratory tract infection around 2-3 weeks prior.

Question 29

What group of bacteria are commonly linked to HSP

Answer 29

Group A Streptococcus

Question 30

What is HSP

Answer 30

``` Generalized small vessel vasculitis affecting the: Skin GI Tract Kidneys Joints. ```

Question 31

Where does HSP rarely affect?

Answer 31

The lungs

Question 32

How does HSP present?

Answer 32

Purpuric rash over buttocks and lower limbs. Abdominal pain D+v Arthralgia

Question 33

Does HSP require treatment?

Answer 33

Its usually self limiting treatment is usually supportive.