Isabelle Kone-Paut1, Rolando Cimaz2, Jethro Herberg3, Oliver Bates3, Aurelia Carbasse4, Jean Pierre Saulnier5, Maria Cristina Maggio6, Jordi Anton7, Maryam Piram8. 1. Université Paris Sud-Saclay, UVSQ, Le Kremlin Bicêtre, France; AP-HP, CHU de Bicêtre, Pediatric Rheumatology, CEREMAIA, Le Kremlin Bicêtre, France. Electronic address: isabelle.kone-paut@aphp.fr. 2. Ospedale Pediatrico Anna Meyer, Pediatric Rheumatology, Firenze, Italy. 3. Imperial College London, Pediatrics, London, United Kingdom. 4. CHU de Montpellier, Pediatrics, Montpellier, France. 5. CHU de Poitiers, Intensive Care Unit, Poitiers, France. 6. University Department Pro.Sa.M.I.G d'Alessandro, Palermo, Italy. 7. Hospital Sant Joan de Déu, Universitat de Barcelona, Pediatric Rheumatology, Barcelona, Spain. 8. Université Paris Sud-Saclay, UVSQ, Le Kremlin Bicêtre, France; AP-HP, CHU de Bicêtre, Pediatric Rheumatology, CEREMAIA, Le Kremlin Bicêtre, France.
Abstract
OBJECTIVES: To identify the clinical characteristics, reasons for use and response to treatment with anakinra in a series of patients with Kawasaki Disease (KD). STUDY DESIGN: A retrospective chart review of patients treated with anakinra for KD diagnosed according to the AHA criteria. We compared clinical, biological and echocardiographic characteristics of KD before and after anakinra use. We analysed reasons for use of anakinra, and compared treatment regimens used in 7 European KD referral centres. RESULTS: Eight boys and 3 girls with treatment-refractory KD, aged 4 months to 9 years old, received at least 2 different KD treatments prior to anakinra, which was given on mean at 25 days after disease onset (8 to 87 days). The main reasons for use of anakinra were clinical and biological inflammation, progression of coronary dilatations, and severe myocarditis with cardiac failure. Doses of anakinra ranged from 2 to 8 mg/kg and duration varied from 6 to 81 days. Efficacy of anakinra was judged in terms of fever resolution (100%), decrease of CRP (100%), and in terms of its effect on coronary artery dilatation Z scores, which decreased in 10/11 patients and increased in one who died suddenly of pericardial hemorrhage. CONCLUSION: Anakinra used late in the disease course led to a rapid and sustained improvement in clinical and biological inflammation. Our retrospective analysis did show neither a striking nor a rapid decrease of coronary dilatations and we cannot determine if anakinra itself had an effect on coronary artery dimensions.
OBJECTIVES: To identify the clinical characteristics, reasons for use and response to treatment with anakinra in a series of patients with Kawasaki Disease (KD). STUDY DESIGN: A retrospective chart review of patients treated with anakinra for KD diagnosed according to the AHA criteria. We compared clinical, biological and echocardiographic characteristics of KD before and after anakinra use. We analysed reasons for use of anakinra, and compared treatment regimens used in 7 European KD referral centres. RESULTS: Eight boys and 3 girls with treatment-refractory KD, aged 4 months to 9 years old, received at least 2 different KD treatments prior to anakinra, which was given on mean at 25 days after disease onset (8 to 87 days). The main reasons for use of anakinra were clinical and biological inflammation, progression of coronary dilatations, and severe myocarditis with cardiac failure. Doses of anakinra ranged from 2 to 8 mg/kg and duration varied from 6 to 81 days. Efficacy of anakinra was judged in terms of fever resolution (100%), decrease of CRP (100%), and in terms of its effect on coronary artery dilatation Z scores, which decreased in 10/11 patients and increased in one who died suddenly of pericardial hemorrhage. CONCLUSION: Anakinra used late in the disease course led to a rapid and sustained improvement in clinical and biological inflammation. Our retrospective analysis did show neither a striking nor a rapid decrease of coronary dilatations and we cannot determine if anakinra itself had an effect on coronary artery dimensions.
Authors: Lauren A Henderson; Scott W Canna; Kevin G Friedman; Mark Gorelik; Sivia K Lapidus; Hamid Bassiri; Edward M Behrens; Anne Ferris; Kate F Kernan; Grant S Schulert; Philip Seo; Mary Beth F Son; Adriana H Tremoulet; Rae S M Yeung; Amy S Mudano; Amy S Turner; David R Karp; Jay J Mehta Journal: Arthritis Rheumatol Date: 2021-02-15 Impact factor: 10.995
Authors: Joseph R Berger; Clyde Markowitz; Peter A Merkel; Rachel A Kolster; Zissimos Mourelatos Journal: J Neurol Date: 2021-02-26 Impact factor: 6.682