Early anti IL-1 treatment replaces steroids in refractory Kawasaki disease: clinical experience from two case reports

Abstract

Refractory Kawasaki disease (KD) is related to a major risk of coronary arteries abnormalities and its treatment is not standardized. In this regard, anakinra (ANA), an interleukin (IL)-1 receptor antagonist, represents an emerging therapeutic option. We report two cases of children, diagnosed with KD, nonresponsive to two doses of intravenous immunoglobulins, successfully treated with ANA, without a prior use of steroids. Patient 2 developed a coronary dilatation, that improved significantly after ANA therapy. Our experience highlights IL-1 blockade effectiveness in reducing KD inflammation and suggests ANA adoption as second-line therapy, with a timesaving and steroid-sparing strategy. Our results, combined with the evidence of the IL-1 key role in KD and coronary arteritis pathogenesis and to the recent clinical evidence reported by the KAWAKINRA trial, encourage an earlier recourse to ANA in patients with refractory KD, in order to fight inflammation, and to treat and prevent the development of coronary artery aneurysms. Further studies are needed to better define the place of IL-1 blockade in KD step-up treatment.

The role of interleukin 1 in the development of human diseases: focus on Anakinra (IL-1 receptor antagonist)

“…Болезнь Кавасаки Ретроспективное исследование, клинические наблюдения [226][227][228][229][230][231][232][233][234][235][236][237][238] Вторичный синдром активации макрофагов/ГЛГ при ИВЗ Клинические наблюдения [239][240][241][242][243][244] COVID-19 ассоциированный гипервоспалительный синдром РПКИ (CORIMUNO, SAVE-MORE) [245,246], наблюдательные исследования [247][248][249][250][251][252][253][254], метаанализы [255,256]…”

Anti-inflammatory Treatment of Kawasaki Disease: Comparison of Current Guidelines and Perspectives

“…Authors concluded that anakinra may be considered as an option after the failure of the first IVIG infusion, especially in patients with coronary involvement ( 90 ). Mastrolia MV et al have recently reported two cases of children, diagnosed with KD, non-responsive to two doses of intravenous immunoglobulins, successfully treated with ANA, without prior use of steroids ( 91 ). Further studies are planned/ongoing to reveal its clinical significance (ANACOMP, ANAKID) and to better define the place of IL-1 blockade in KD step-up treatment.…”