Vasculitis

Question 1

What is vasculitis?

Answer 1

inflammation of the blood vessels (arteries, arterioles, veins, venules and capillaries)

Question 2

What does primary vasculitis result from?

Answer 2

results from an inflammatory response that targets the vessel walls, can be autoimmune

Question 3

What does secondary vasculitis result from?

Answer 3

triggered by an infection, drug or toxin and can occur as part of an inflammatory disorder or cancer

Question 4

What is large vessel vasculitis?

Answer 4

primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches

Question 5

What are the two subtypes of LVV?

Answer 5

Giant cell arthritis

Takayasu arthritis

Question 6

What populations does Takayasu arthritis affect?

Answer 6

East Asian populations - some western

Middle aged women - 20-30yrs

Question 7

What populations does Giant cell arthritis affect?

Answer 7

over 50s

Question 8

What does Giant cell arthritis cause?

Answer 8

temporal arteritis

Question 9

What are the main signs of LVV?

Answer 9

Bruit 
claudication
headaches
BP difference of extremities
unilateral temperal headache
jaw claudication
scalp tenderness
risk of blindness
polymyalgia rheumatica

Question 10

What are the investigations for LVV?

Answer 10

ESR, plasma viscosity
increased CRP
temporal artery biopsy
angiogram
PET

Question 11

What is the management for LVV?

Answer 11

40-60mg prednisolone then gradually decrease

use steroid sparing agent (methotrexate) if necessary

Question 12

What is the histological sign of LVV?

Answer 12

granulomatous infiltraiton of the walls of the large vessels

Question 13

What are the different subgroups of Small vessel vasculitis?

Answer 13

ANCA positive

ANCA negative

Question 14

What are the ANCA positive SVV?

Answer 14

GPA - granulomatosis with polyangitis
EGPA - eosinophillic granulomatosis with polyangitis
Microscopic polyangitis

Question 15

What is EGPA also called?

Answer 15

Chaug-Strass Syndrome

Question 16

What does GPA comprise of?

Answer 16

granulomatous inflammation of the respiratory tract, small and medium vessels
necrotising glomonephritis is also common

Question 17

What is GPA associated with?

Answer 17

cANCA and PR3

Question 18

What are the symptoms of GPA?

Answer 18

nose bleeds
deafness
recurrent sinusitis
nasal crusting (over time there can be collapse of the nose)
haemoptysis 
cavitating lung lesions on x-ray

Question 19

What is EGPA characterised by?

Answer 19

same as GPA, but with late onset asthma, rhinitis, and increased eosinophil count
mono neuritis multiplex can also occur

Question 20

What is EGPA associated with?

Answer 20

pANCA

Question 21

What is Microscopic polyangitis characterised by?

Answer 21

necrosing vasculitis

90% get glomornephritis

Question 22

What is MP associated with?

Answer 22

pANA and MPO

Question 23

How do you test for an ANCA?

Answer 23

immunofluresence

Question 24

What can cause ANCA positive conditions?

Answer 24

HLA genese, CD226 and IL-10
silica
Staph. aureus, E.coli, K. pneumoniae
Drugs - propylthiouracil, hydralazine, minocycline

Question 25

How is EGPA diagosed?

Answer 25

eosinophilia of more than 10% of blood

Question 26

What is the pathophysiology of ANCA positive conditions?

Answer 26

associated with immune complexes so complement is consumed meaning C3/C4 may fall

Question 27

What is the management for ANCA positive conditions?

Answer 27

IV steroids and cyclophosphamide when aggressive
if localised/early - methotrexate and steroids
if creatinine is >500 - plasma exchange
if refractory - IV immunoglobulins and Rituximab

Question 28

What are the investigations for ANCA positive conditions?

Answer 28

ESR, PV and CRP and raised
Anaemia of chronic disease is common.
U+E looking for renal involvement
Anti-neutrophil cytoplasmic antibody (ANCA)
Urinalysis (looking for renal vasculitis)
CXR
Biopsy of an affected area e.g. skin or kidney is often helpful in confirming the diagnosis

Question 29

What is the ANCA negative condition?

Answer 29

Henoch-Schönlein purpura (HSP)

Question 30

Desribe Henoch-Schönlein purpura (HSP)?

Answer 30

IgA mediated disorder of generalised vasuclitis affecting the small vessels of the skin, GI tract, kidneys, joints, lungs and CNS

Question 31

What populations does HSP commonly affect?

Answer 31

children ages 2-11

75% patients have a proceeding URTI, pharangeal infection or GI infection (normally staph aureus)

Question 32

What are the common symptoms for HSP?

Answer 32

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

Question 33

What is the management of HSP?

Answer 33

symptoms resolve within 8 weeks normally

need to do renal analysis to check for renal involvement