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Are TNF‐α blockers effective and safe for Kawasaki disease in children? A Cochrane Review summary with commentary

Kawasaki disease (KD) is an acute vasculitis with unknown etiology affecting smalland medium-sized blood vessels, particularly coronary arteries, which usually occurs in early childhood up to 5 years of age.2 This condition is characterized by main clinical features including fever, swelling of cervical lymph nodes, edema or erythema of the extremities, arthritis, and mucocutaneous manifestations such as congestion of the ocular conjunctivae, and oropharyngeal swelling and reddening.3 KD has a particularly high incidence in Japanese children (264.8 per 100 000), even though it has been reported worldwide, with a male-to-female ratio of 1.5:1.4 Coronary artery abnormalities (CAAs: from dilation to aneurysm that can result in coronary artery thrombosis) are the primary cause of morbidity and mortality in patients with KD. Despite the fact that pathogenesis remains poorly understood, some mechanisms have been described, including endothelial cell activation due to increased immune response recruiting neutrophils, macrophages, lymphocytes, plasma cells, and eosinophils, as well as enhanced expression of key proinflammatory cytokines triggering vasculitis, such as interleukin (IL)1, IL-6 and tumor necrosis factor (TNF)-α.5 From a functional perspective, patients might experience deconditioning after the acute phase of KD because of CAAs and impaired coronary perfusion that influence aerobic capacity and exercise performance.6 Moreover, other functional impairments, such as facial palsy, sensorineural hearing and visual loss, ataxia, as well as behavioral disorders, have been described. These complications might result in potentially irreversible functional limitations in activities of daily living and poor health-related quality of life.7-11 The main target of the acute phase of KD is suppression of inflammation and prevention of CAAs. According to available evidence, early administration of intravenous immunoglobulin (IVIG) at high doses combined with acetylsalicylic acid is the first-line treatment for KD.5,12 However, up to 20% of patients do not respond to this therapy, requiring further interventions to prevent cardiovascular complications. Several biologics targeting inflammatory cytokines have been proposed to treat the IVIG-resistant population, including monoclonal antibodies against TNF-α because of emerging evidence about their role in reducing endothelial cell apoptosis and CAAs. However, the efficacy and safety of TNF-α in KD patients remain poorly investigated. This Cochrane Systematic Review (CSR) summarizes the available evidence about one of the available treatments for IVIG-resistant KD cases that might improve management strategies to prevent severe functional limitations in children who will become adults with disabilities.