[Rheum] Vasculitis

Question 1

what is vasculitis?

Answer 1

a group of autoimmune diseases that affect the vessels

Question 2

what can vasculitis be classified as?

Answer 2

• large vessel disease
• medium vessel disease
• small vessel disease

Question 3

how does vasculitis present?

Answer 3

multiple organ systems are affected so pts present as polysymptomatic

Question 4

what should polysymptomatic pts have?

Answer 4

rheumatology work up with:

• ANA (anti-nuclear antibodies)
• ANCA (anti-neutrophilic cytoplasmic antibodies)

Question 5

what should be done if ANA or ANCA came back +ve?

Answer 5

further rheumatological ix:
- other antibodies
+/- biopsy (gold standard) of kidney/skin

Question 6

what is the ANCA associated with granulomatosis with polyangiitis (GPA)?

Answer 6

c-ANCA

Question 7

what are the defining sx of granulomatosis with polyangiitis (GPA)?

Answer 7

• saddle shaped nose

- recurrent epistaxis / sinusitis

Question 8

what does histology for granulomatosis with polyangiitis (GPA) show?

Answer 8

granulomas

Question 9

what is the ANCA associated with microscopic polyangiitis?

Answer 9

p-ANCA

Question 10

what are the defining sx of microscopic polyangiitis?

Answer 10

similar to GPA but less likely to have nasopharyngeal involvement

Question 11

what does histology for microscopic polyangiitis show?

Answer 11

no granulomas

Question 12

what is the ANCA associated with eosinophilic granulomatosis with polyangiitis (EGPA)?

Answer 12

p-ANCA

Question 13

what are the defining sx of eosinophilic granulomatosis with polyangiitis (EGPA)?

Answer 13

asthmatic, quite often requires intense rx for control

Question 14

what does histology for eosinophilic granulomatosis with polyangiitis (EGPA)?

Answer 14

granulomas

Question 15

what are the common Sx shared by GPA, microscopic polyangiitis and EGPA?

Answer 15

• renal impairment / rapidly progressive GN (RPGN)
• lung inflammation and haemoptysis
• skin ulceration (purpura in lower limbs)

Question 16

what are the common Rx shared by GPA, microscopic polyangiitis and EGPA?

Answer 16

• rx remission: cyclophosphamide and corticosteroids

- once remission: low dose steroids + methotrexate / mycophenolate / azathoprine

Question 17

what is the most common large vessel vasculitis?

Answer 17

Giant Cell Arteritis (GCA)

Question 18

what arteries does GCA affect?

Answer 18

large arteries in the head, most commonly temporal artery

Question 19

in whom is GCA rare in?

Answer 19

<50 year olds

Question 20

what are the sx of GCA?

Answer 20

• single sided temporal headaches
• scalp and jaw tenderness (painful shaving, combing hair)
• visual disturbance: ophthalmic artery involvement [ophthalmic emergency]

Question 21

what should be given urgently in GCA?

Answer 21

prednisolone (40-60mg)

60-100mg if visual sx

Question 22

what is the gold standard dx for GCA?

Answer 22

temporal artery biopsy

Question 23

heavy smoker, recurrent digit ischaemia. dx?

Answer 23

thromboangiitis obliterans (Buerger’s)

Question 24

haemoptysis, haematuria and anti-GBM. dx?

Answer 24

Goodpasture’s syndrome

Question 25

new agitation and hallucinations on prednisolone. dx?

Answer 25

steroid induced psychosis

Question 26

fever >5 days, conjunctivitis, strawberry tongue. dx?

Answer 26

Kawasaki's disease

Question 27

transmural inflammation, "beads on a string" angiography. dx?

Answer 27

polyarteritis nodosa

Question 28

recurrent mouth and genital ulcers not due to infection. dx?

Answer 28

Behcet syndrome

Question 29

what should all small vessel disease be treated with initially?

Answer 29

cyclophosphamide and corticosteroids

Question 30

what should be performed if there is renal, lung or skin involvement?

Answer 30

biopsy

Question 31

what is the common autoantibody present in the following: - rheumatoid arthritis - Sjögren's syndrome?

Answer 31

rheumatoid factor (RF)

Question 32

what is the autoantibody present in rheumatoid arthritis?

Answer 32

anti-CCP

Question 33

what is the common autoantibody present in the following? - SLE - systemic sclerosis (limited and diffuse) - Sjögren's syndrome - poly-/dermato-myositis - autoimmune hepatitis

Answer 33

ANA

Question 34

what are the autoantibodies present in SLE?

Answer 34

anti ds-DNA and anti-Smith (Sm)

Question 35

what is the autoantibody present in limited systemic sclerosis?

Answer 35

anti-centromere

Question 36

what is the autoantibody present in diffuse systemic sclerosis?

Answer 36

anti-Scl-70

Question 37

what are the autoantibodies present in Sjögren's syndrome?

Answer 37

anti-Ro and anti-La

Question 38

what are the autoantibodies present in poly-/dermato-myositis?

Answer 38

anti-Jo and anti-Mi2

Question 39

what is the autoantibody present in drug induced lupus?

Answer 39

anti-histone

Question 40

what is the autoantibody present in vasculitides?

Answer 40

ANCA