Anakinra for Treatment-Resistant Kawasaki Disease: Evidence from a Literature Review
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REVIEW ARTICLE
Anakinra for Treatment‑Resistant Kawasaki Disease: Evidence from a Literature Review Giovanna Ferrara1 · Teresa Giani2,3 · Maria Costanza Caparello5 · Carla Farella2 · Lisa Gamalero4 · Rolando Cimaz5,6
© Springer Nature Switzerland AG 2020
Abstract Kawasaki disease (KD) is one of the most common vasculitides of childhood and the main cause of acquired heart disease in developed countries. Intravenous immunoglobulin (IVIG) in association with aspirin represents the main treatment for KD. However, 10–20% of patients fail to respond to standard treatment and have an increased risk of cardiac complications. There is currently no accepted protocol for treatment of resistant cases. Several authors highlighted the role of interleukin-1 (IL-1) as a mediator of inflammation in KD and suggested the possibility of using IL-1 or its receptor as a target of therapy. The use of IL-1 inhibitors in patients with KD has been reported in the scientific literature, but data are largely limited to individual case reports and small case series. We summarized the scientific literature related to the use of anakinra, analyzing preclinical and clinical data. Thirty-eight patients have been described so far, most of them with KD-related complications. Twenty-two were described in case reports and case series, while 16 were patients from the completed KAWAKINRA phase IIa study. Almost all patients received clinical benefit, and no relevant side effects were noted. Based on this evidence, in our opinion, anakinra may be considered as an option after the failure of the first IVIG infusion, especially in patients with coronary involvement. Key Points Interleukin-1 (IL-1) plays a key role in the pathogenesis of Kawasaki disease (KD), especially in the development of coronary artery aneurysms. Based on current evidence, derived from case reports and one open-label, phase II study, the IL-1 inhibitor anakinra is a fast-acting, effective, and reasonably safe drug. Anakinra may be considered as an option to treat KD resistant to a first intravenous immunoglobulin (IVIG) dose, especially in the presence of severe coronary involvement. * Teresa Giani [email protected] 1
ASL Toscana Centro, Florence, Italy
2
AOU Meyer, Florence, Italy
3
Department of Medical Biotechnology, University of Siena, Siena, Italy
4
University of Udine, Udine, Italy
5
ASST G-Pini, Milan, Italy
6
Department of Clinical Sciences and Community Health and RECAP‑RD, University of Milan, Milan, Italy
1 Introduction Kawasaki disease (KD) is an acute medium vessel vasculitis of childhood, typically involving coronary arteries, and represents in this age group the first cause of acquired heart disease in developed countries. Classically, KD is diagnosed in the presence of a high fever lasting for at least 5 days associated with at least four of the five principal features: changes in the peripheral extremities, cervical lymphadenopathy, bilateral non-exudative conjunctivitis, changes in the oral cavity, and polymorphous exanthema [1]. In t
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