Vasculitis

Question 1

What is vasculitis?

Answer 1

Presence of leukocytes/immune complexes in the vessel wall with reactive damage to mural structures

Question 2

Examples of Lagre vessel vasculitis

Answer 2

Takayasu artheritis

Giant Cell arteritis

Question 3

Examples of Medium vessel vasculitis

Answer 3

PAN
Kawasaki’s
Isolated CNS vasculitis

Question 4

Examples of Small vessel vasculitis

Answer 4

Churg-Strauss
Wegner’s
Microspcopic polyangitis
any many more- most well known ones

Question 5

What are possible ways to classify

Answer 5

Size

ANCA neg or pos

Question 6

What percentage Wegner’s-Granulomatosis with Polyangitis are ANCA positive vasculitides?

Answer 6

90%

Question 7

What are mimickers of vasculitis?

Answer 7

Infectious diseases
Drug toxicity
Thrombotic microangiopathies
Neoplasms
Miscellaneous

Question 8

What is essential for diagnosis of vasculitis?

Answer 8

Skin biopsy of the area affected

ANCA good but not diagnostic as it does not occur in all

Question 9

What can be used to assess patients in clinic to discover how extensive it is

Answer 9

BVAS (Birmingham vasculitis score)

Question 10

Treatment

Answer 10

Based on how extensive
Localised/early systemic - methotrexate and steroids/ azathioprine and steroids
Generalised/systemic - cyclophosphamide and steroids (1st line) Rituximab and steroids (alternative)
Plasma exchange if creatine >500
Followed by azathioprine with alternatives being methotrexate, mycophenolate mefetil or leflunomide

Refractory-IV immunoglobulins
-Rituximab

Question 11

Whats the cut off creatine for what can be described as life threatening?

Answer 11

500

Question 12

If untreated… but if treated

Answer 12

usually fatal

Treated- 5 year survival is 90%

(but mortality in treated patients 2.6 times higher)