Recent advances in the treatment of Kawasaki disease

Abstract

Kawasaki disease (KD) is acute systemic vasculitis that occurs mainly in infants and children under 5 years of age. The etiology of KD remains unknown. KD is liable to be complicated by coronary artery lesions (CALs), which develop in approximately 15-25% of untreated KD children and in approximately 5% of KD children after intravenous immunoglobulin (IVIG) therapy. A single high dose of IVIG (2 g/kg) is the gold standard therapy in the acute stage of KD. However, approximately 8--38% of children are unresponsive to initial IVIG treatment and at increased risk for CAL development. Anti-inflammatory high doses of aspirin are recommended in conjunction with IVIG, but our study demonstrated that there is no evidence of efficacy in preventing CAL development. The usefulness of steroids in initial therapy for KD or treatment of IVIG-resistant patients is not well established. Other immunosuppressive therapies, including infliximab, have been used in the treatment of refractory KD, but merit additional investigation. Subclinical atherosclerosis may develop early in KD patients, which makes early initiation of therapy to improve chronic inflammation an important issue. Future multicenter studies may help to define the optimal management of KD patients.

Kawasaki disease: etiopathogenesis and novel treatment strategies

“…Chen et al [72] performed a meta-analysis of clinical trials to compare the efficacy of IVIG plus steroid treatment compared to IVIG alone and concluded that their combined therapy as an initial treatment in KD is more useful in reducing the risk of CAA. IVMP through their anti-inflammatory functions suppress cytokine levels faster than IVIG alone [73]. The usefulness of second dose of IVIG versus steroids in IVIG resistant cases needs to be fully established [14].…”

Recent Understanding on Diagnosis and Management of Central Nervous System Vasculitis in Children

“…Randomized controlled trials have shown that a single infusion of 2 g/kg of IVIG given 5–10 days after the onset of fever, eliminated fever in 85–90% of children within 36 h and significantly reduced the risk of CALs [24]. Five possible mechanisms include Fc receptor blockade, neutralization of the causative agents, or a toxin produced by an infectious agent, an immunomodulating effect, induction of suppressor activity, and modulation of the production of cytokines and cytokine antagonists [25]. …”

Refractory, missed and severe Kawasaski disease: Diagnostic and therapeutic challenges

“…It is clear that further investigation of the role of infliximab in IVIGresistant patients is needed, especially as recognition of this condition improves and more patients are diagnosed timeously. (24) Results are awaited from a recently completed large multi-centre study in the USA comparing standard therapy with standard therapy plus infliximab as initial therapy for all patients with KD. (25) Patients not responding to these agents may be considered for other TNF-α blockade therapy.…”