Blog
About

13
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      The effect of Anakinra on Quality-of-Life in a family with colchicine-resistant FMF

      , 1 , 2 , 1

      Pediatric Rheumatology Online Journal

      BioMed Central

      8th International Congress of Familial Mediterranean Fever and Systemic Autoinflammatory Diseases

      30 September-3 October 2015

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Introduction About 5-10% of patients with FMF do not to colchicine treatment to control inflammation. In these patients anti-IL1 therapy seems to be effective in controlling inflammation, although clinical trials are lacking. However, many patients on Anakinra experience side effects including headache and injection-site reactions. How Anakinra treatment influences quality-of-life (QoL) in these patients is unknown. Objectives To investigate the effect of Anakinra on inflammation and QoL in a family with a severe phenotype colchicine-resistant FMF. Patients and methods Four sisters of Italian descent with homozygote M694V-positive FMF, had an extraordinary phenotype with continuous inflammation, daily fevers, erysipelas-like skin lesions and serositis. Colchicine was initiated in all patient, but showed ineffective in controlling inflammation. Eventually, Anakinra was initiated. Before and during treatment with Anakinra inflammatory parameters (CRP, leukocyte-count, Serum amyloid A) were measured. One patient only started Anakinra after type AA amyloidosis had developed leading to a renal replacement therapy and renal transplantation. In the other three patients QoL questionnaire (RAND-36) was obtained, before and 4 weeks after start of Anakinra-therapy. The study was approved by the local ethical committee. Because of reimbursement was initially declined, Anakinra was discontinued. Later, it could be re-initiated. QoL questionnaires were obtained after stopping and after re-initiation of Anakinra. Results During Anakinra treatment the CRP values were significantly lower than without Anakinra (mean 3.7 mg/L vs 30 mg/L, p<0.001). The QoL improved significantly after initiation of Anakinra in 5 out of 6 scales of the RAND-36 (p<0.05). The QoL deteriorated after withdrawal of Anakinra to pre-treatment level and improved again after re-initiation of Anakinra. Conclusion Here we show that Anakinra can effectively suppress inflammation in a family with severe colchicine-resistant FMF. This lead to a significant improvement in quality of life.

          Related collections

          Author and article information

          Conference
          Pediatr Rheumatol Online J
          Pediatr Rheumatol Online J
          Pediatric Rheumatology Online Journal
          BioMed Central
          1546-0096
          2015
          28 September 2015
          : 13
          : Suppl 1
          : P108
          Affiliations
          [1 ]Jessa Hospital, Infectious Diseases and Immunity, Hasselt, Belgium
          [2 ]University of Hasselt, BIOMED, Hasselt, Belgium
          1546-0096-13-S1-P108
          10.1186/1546-0096-13-S1-P108
          4599955
          Copyright © 2015 van der Hilst and Lijnen

          This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

          8th International Congress of Familial Mediterranean Fever and Systemic Autoinflammatory Diseases
          Dresden, Germany
          30 September-3 October 2015
          Categories
          Poster Presentation

          Pediatrics

          Comments

          Comment on this article