Page 312 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

286 Dimitri Chanouzas and Matthew David Morgan

disease can be managed successfully with open revascularization [10]. Endovascular
management employing percutaneous trans-catheter embolization is being used with
increasing frequency to deal with ruptured visceral artery aneurysms associated
with PAN [62].

Kawasaki Disease

Epidemiology and Clinical Features
Kawasaki disease (KD) is one of the commonest childhood vasculitides with a

particularly high incidence and prevalence in East Asia. Most patients have disease onset
before 5 years of age with a peak incidence at 30 months [63]. The diagnosis is confirmed by
the presence of fever for > 5 days, the presence of four of the following five diagnostic
criteria including, bilateral conjunctival injection, changes of the mucous membranes of the
upper respiratory tract including injected pharynx, fissured lips or strawberry tongue;
polymorphous rash; limb changes including peripheral edema, erythema, or periungual
desquamation; and cervical adenopathy. Untreated KD is a self-limiting disease that lasts for
10 to 12 days; however, coronary artery aneurysms develop in 20% to 25% of patients with
significant associated morbidity and mortality [63].

Gastrointestinal Involvement
Gastrointestinal involvement is relatively uncommon but tends to involve the gallbladder

and the liver leading to acute non-calculous distension of the gallbladder (gallbladder
hydrops) as well as hepatic dysfunction with serum transaminase elevations [64, 65].
Presentation with an acute abdomen was noted in 4.6% of children with the diagnosis of KD
[65] due to vasculitic appendicitis, paralytic ileus, or hemorrhagic duodenitis.

Treatment
Treatment of KD with aspirin and intravenous immunoglobulin (IVIg) reduces the

incidence of coronary artery complications compared with aspirin alone [66].

Small Vessel Vasculitides

Involvement of small vessels by vasculitis can lead to ulceration, erosions, strictures and
occasionally perforation.

IgA Vasculitis/Henoch-Schönlein Purpura

Epidemiology and Clinical Features
Henoch-Schönlein Purpura or IgA vasculitis (IgAV/HSP) is a small vessel vasculitis

characterized by a leukocytoclastic vasculitis and deposition of IgA-containing immune
complexes (IC). It is the commonest form of systemic vasculitis in children with a peak
incidence at four to six years of age, although adult patients have been described [67].

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