Interleukin-1 antagonists in Mevalonate Kinase Deficiency
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POSTER PRESENTATION
Open Access
Interleukin-1 antagonists in Mevalonate Kinase Deficiency C Galeotti1*, L Rossi-Semerano1, S Guillaume1, A Duquesne2, P Pillet3, O Richer3, E Hachulla4, I Koné-Paut1 From 18th Pediatric Rheumatology European Society (PReS) Congress Bruges, Belgium. 14-18 September 2011 Background Mevalonate Kinase Deficiency (MKD) is one of the autoinflammatory fever syndromes, caused by mutations in the MKD gene. Systemic inflammatory symptoms may be mild to severe leading to early death, and recurrent bacterial infections frequently develop in the disease course. On demand NSAID and steroids are the most commonly used. Few case-reports suggested that interleukin-1 (IL-1) antagonists could alleviate MKD symptoms. Aim We describe efficacy and safety of IL-1 targeting drugs, anakinra and canakinumab, in seven children with MKD. Methods A questionnaire containing items of disease activity was sent to French pediatric and adult rheumatologists. We collected information from 7 MKD patients treated with IL-1 antagonists. MKD was suspected clinically then confirmed genetically in all cases. Results The use of IL-1 antagonists was beneficial to all patients on a long term. Anakinra was daily administered and canakinumab every 8 weeks, with dose adjustments. The median clinical score based on fever and symptoms during MKD crisis before and after treatment was 9 and 0,5 respectively. The number of days of fever during crisis before and after treatment was 5,25 and 1,75 respectively. These treatments alleviated biological inflammatory markers. The side effects with anakinra were local
pain, inflammatory signs at the injection site, infections (bacterial pneumopathy and rhinopharyngitis) and acne. There was no side effect with canakinumab. Three patients switched from anakinra to canakinumab.
Conclusion IL-1 targeting drugs appear effective and safe in our MKD patients. Controlled trials to further assess their clinical benefit and safety in MKD patients seem necessary. Author details 1 Department of Pediatric Rheumatology, CEREMAI, CHU Bicêtre, university of Paris Sud, France. 2Department of Pediatric Rheumatology, CHU Lyon, France. 3Department of Pediatric Rheumatology, CHU Bordeaux, France. 4 Department of Internal Medicine, CHRU Lille, France. Published: 14 September 2011
doi:10.1186/1546-0096-9-S1-P7 Cite this article as: Galeotti et al.: Interleukin-1 antagonists in Mevalonate Kinase Deficiency. Pediatric Rheumatology 2011 9(Suppl 1):P7.
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* Correspondence: [email protected] 1 Department of Pediatric Rheumatology, CEREMAI, CHU Bicêtre, university of Paris Sud, France Full list of author information is available at the end of the article
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