How is vasculitis treated?

Updated: Dec 10, 2018
  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
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Answer

The management of children with Kawasaki disease involves hospital admission and treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin in the acute phase of the illness. [31] Subsequently, daily low-dose aspirin is given for 6-8 weeks until follow-up echocardiography. IVIG-resistant disease may be treated with methylprednisolone and/or other immunosuppressive therapies (see Kawasaki Disease “Treatment of IVIG-Resistant Disease”)

The management of children with Henoch-Schönlein purpura is primarily symptomatic, and most patients do not require hospital admission. Nonsteroidal anti-inflammatory drugs (NSAIDs) can be given for joint pain or swelling. Corticosteroids may be considered in selected patients (ie, those with severe GI symptoms), [32] but is an area of controversy in the literature. [33] Clinically significant nephritis is typically treated with steroids and other immunosuppressive therapies.

Patients with chronic vasculitides should be managed by a multidisciplinary group of specialists (eg, rheumatologists, cardiologists, nephrologists) and require long-term follow-up for monitoring of relapses, disease activity, end-organ damage and morbidity associated with therapy.

Infliximab and adalimumab can be considered as first-line immunomodulatory agents for the treatment of ocular manifestations of Behçet's disease. [34]

No therapeutic trials have looked at management of vasculitis in the pediatric population, and practice has been based on adult guidelines, which have been summarized. [35, 36, 37] These recommendations provide general guidance that should be modified based on the features of each individual’s illness.


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