Vasculitis (cortex)

Question 1

Inflammation of blood vessels may result in?

Answer 1

Vessel wall thickening
stenosis
occlusion with subsequent ischaemia

Question 2

What does large vessel vasculitis refer to?

Answer 2

Vasculitis that causes chronic granulomatous inflammation

mostly in the aorta and its major branches

Question 3

What are the two subtypes of large vessel vasculitis?

Answer 3

Takasayu arteritis 

Tenporal/giant cell arteritis

Question 4

Which type of large vessel vasculitis
occurs mostly >50yrs old?
occurs mostly <50 yrs old?

Answer 4

> 50yrs old temporal/giant cell arteritis

<50yrs old takasayu arteritis

Question 5

25 Year old japanese woman with malaise, night sweats, weight loss, arthralgia and fatigue
claudicant symptoms in upper and lower limb

Answer 5

Takasayu

Question 6

In GCA ESR PV and CRP are elevated

Answer 6

T

Question 7

If GCA is left untreated what can occur?

Answer 7

Vascular stenosis, aneurysms

resulting in bruit and reduced pulses

Question 8

what is the treatment for large vessel vasculitis?

Answer 8

Prednisolone starting at 40-60mg and gradual reduction

steroid sparing agents such as methotrexate ir azathioprine may be added

Question 9

How can small vessel vasculitis be grouped?

Answer 9

Antineutrophil cytoplasmic antibodies positive or negative

Question 10

Small vessel vasculitis’ conditions have simmilar presenting features

Answer 10

T

Question 11

What are the small vessel vasculitis presenting symptoms?

Answer 11

Fever, Wight loss, raised non blanching purpuric rash. arthralgia/arthritis

Question 12

What are the main complications of small vessel vasculitis?

Answer 12

Pulmonary vasculitis - leading to lung opacities on X ray
mononeuritis multiplex
glomerulonephritis

Question 13

ANCA +ve small vessel vasculitis with no granulomas present

Answer 13

Microscopic polyangitis

Question 14

ANCA +ve small vessel vasculitis with granulomas present

Answer 14

Wegner’s granulomatosis/ polyangitits

Question 15

ANCA +ve small vessel vasculitis with granulomas present and asthma and eosinophilia

Answer 15

Churg-Strauss syndrome

Question 16

Anca -ve vasculitis with IGA

Answer 16

Honoch Schnein purpura

Question 17

Anca -ve with serum crygoglobulin

Answer 17

cryoglobulinaemia

Question 18

What are the features of Wegner’s granulomatosis/ polyangititis?

Answer 18

Granulomatous inflammation 
ENT and Resp symptoms
Necrotising vasculitis of small/medium vessels
glomerulonephritis common
Cavitating lesions on X ray

Question 19

What are the features of Microscopic polyangitis?

Answer 19

Necrotising vasculitis with few or no immune deposits
pulmonary capilliaritis and glomerulonephritis is common
pulmonary caprillitis often occurs

Question 20

What is renal limited vasculitis?

Answer 20

Necrotizing glomerulonephritis with no extra renal features at presentation

Question 21

What is the main difference between Churg Strausss/EGPA syndrome and Wegner’s/granulomatosis with polyangitis?

Answer 21

Churg Strauss is associated with asthma and eosinophilia

Necrotizing glomerulonephritis is more common in W/GPA

Question 22

What are the features of Churg Strass syndrome?

Answer 22

Eosinophil-rich granulomatous inflammation involving resp tract
Necrotizing vasculitis involving small to medium sized vessels
assoc asthma and eosinophilia

Question 23

What are the symptoms of Granulomatosis with polyangiitis or Wegeners?

Answer 23

ENT SYMPTOMS
-nose bleeds
-deafness
-recurrent sinutitis
-nasal crusting 
Resp symptoms 
- Haemoptysis

Question 24

Which antibodies are GPA/Wegners assoc with?

Answer 24

Proteinase 3 antibodies

Cytoplasmic antineutrophil cytoplasmic antibodies

Question 25

What are symptons of churg strauss/ egpa?

Answer 25

late on set asthma | rhinitis/coryza: stuffy nose, sneezing etc.

Question 26

Glomerulonephritis occurs in 90% in patients with which condition

Answer 26

Microscopic polyangiitis

Question 27

ANCA is negative in proportion of egpa, gpa and Mpa so it cannot be relied upon to confirm the diagnosis.

Answer 27

T

Question 28

What investigations should be done in a suspected vasculitis?

Answer 28

``` Bloods - ESR, PV and CRP are raised - Anaemia common -ANCA Renal involvement - urinalysis - U+Es ``` - CXR - Biopsy (skin or kidney)

Question 29

How are ANCA associated vasculitis managed?

Answer 29

IV steroids and cyclophosphamide - a chemotherapy drug

Question 30

What is Henoch-Schonlein purpura?

Answer 30

IgA mediated generalised vasculitis affecting small vessels in skin, GI tract, kidneys, joints

Question 31

Henoch-Schonlein purpura rarely affects lungs and cns

Answer 31

T

Question 32

Henoch-Schonlein purpura commonly affects CHILDREN

Answer 32

T

Question 33

What is the common history of people affected by Henoch-Schonlein purpura?

Answer 33

URTO predates by few weeks

Question 34

What are symptoms of Henoch-Schonlein purpura?

Answer 34

purpuric rash over the buttocks and lower limbs, abdominal pain and vomiting and joint pain.

Question 35

Treatment Henoch-Schonlein purpura?

Answer 35

self limiting condition not requiring specific treatment. It usually settles over the course of weeks to months