Vasculitides

Question 1

Classify vasculitides by vessel size:

Answer 1

LARGE
Giant Cell Arteritis
Takayasu

MEDIUM
Kawasaki
Polyarteritis Nodosa
Thromboangitis Obliterans (Beurger)

SMALL
HSP
Wegener’s
Behcet
Goodpasture
Microscopic polyangitis
Churg-Struass

Question 2

Complications of vasculitides (general):

Answer 2

Thrombosis
Stenosis
Aneurysm
Ischaemia/infarct
—> Incl MI, stroke
End-organ damage:
—> stroke, optic neuropathy, renal failure, neuropathies etc.
Aneurysm
—> Incl. AAA
Rupture/ bleeds

Complications of therapy

Question 3

Age affected by Kawasaki:

Answer 3

<5 years (80%)

Peaks 18-24 mo

Question 4

Diagnostic criteria for Kawasaki:

Answer 4

Fever 5 days**
PLUS
4 of:
-Red eyes (conjunctival injection)
-Red rash (variable)
-Red mouth
—> Strawberry tongue
—> Cracked lips
—> Red pharynx
-Red and swollen hands/feet
—> Painful oedema
—> Progresses to desquamation
-Lymphadenopathy (unilateral)
—> At least 1 >1.5cm
_____________________________

Lab results support, but diagnosis clinical.

Question 5

Cardiac complications of Kawasaki:

Answer 5

Leading cause of paediatric heart disease in developed world

Coronary artery aneurysm-
*25% if untreated! —> MI
—> Age extremes
—> More common in incomplete KS
Pericarditis
Myocarditis
Pericardial effusion
LV dysfunction
Valvular dysfunction

Question 6

Treatment of Kawasaki:

Answer 6

IVIG 2g/kg as single dose on diagnosis

ASPIRIN 5mg/kg daily

Paeds may consider steroid in high risk/ refractory

Echo- serial

Question 7

Platelets in Kawasaki:

Answer 7

Typically rise (extremely) after 1 week

Question 8

Diagnostic criteria for incomplete Kawasaki:

Answer 8

Even higher risk for coronary aneurysm!

Typically <1yo

-Infant with fever >7days
- Any age, fever >5 days and at least TWO features

AND must have some supportive investigations
-CRP/ESR/WCC up
-Plts up after 7d
-Low albumin
-High ALT
-WCC in urine

-Abnormal ECG or echo

Question 9

Age affected by HSP:

Answer 9

2-8 years

Can also be adults- more severe

Question 10

Clinical features of Henoch-Schonlein Purpura (HSP):

Answer 10

TYPICAL TRIAD:
Palpable purpura
—> dependent areas
—> +- petechiae, ecchymosis
Abdominal pain
—> colicky
—> small GI bleed
—> BAD: intussusception, ischaemia.
Arthritis
—> Knees, ankles. Migratory.

Plus:
RENAL
—> haematuria, proteinuria
—> Nephrotic, nephritic
—> HTN, AKI
*** 15% get permanent renal failure.

…but can affect anything.

Question 11

Which tests MUST be done in suspected HSP?

Answer 11

Blood pressure
Urinalysis

Question 12

Management of HSP:

Answer 12

If:
-Well
-Blood pressure normal
-Urinalysis normal (micro haematuria in isolation okay)
—> Can be discharged
—> Check BP and urinalysis weekly

Steroids if joint pain severe + unresponsive to simple analgesia.
Eg. 1-2mg/kg Pred daily.
Symptomatic role only.

Immunomodulation if nephritis.

25% recurrence

Question 13

Giant Cell Arteritis: clinical features

Answer 13

Most common vasculitis

Whites
Age >50
ESR >50
Tx with 50 of pred

Aorta —> carotids —> cranial and eye branches

CLINICAL
Fever
Unilateral headache (85%)
Painless, unilateral vision loss (50%) —> anterior ischaemic optic neuritis
Jaw claudication -Most specific
Painful scalp
Tender/ ropey-next most specific/ pulseless temporal artery
Ocular: pale, swollen optic disc, RAPD Overlap with PMR

Question 14

Giant Cell Arteritis: management

Answer 14

MANAGEMENT:
1-Steroid:
—> Vision okay: Prednisolone 1mg/kg PO
—> Vision loss: admit for IV methylpred, 1g QID for 3 days
2- Aspirin
3- Refer for biopsy within a few days
—> Vasc/ ophthal
—> (negative doesn’t exclude)