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Etanercept and anakinra can prolong febrile episodes in patients with hyperimmunoglobulin D and periodic fever syndrome

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Abstract

Hyperimmunoglobulin D and periodic fever syndrome (HIDS) is a rare, hereditary autoinflammatory condition, characterized by recurrent inflammatory episodes. There is no proven treatment for HIDS, but various drugs including, non-steroidal anti-inflammatory drugs, colchicine, steroids, statins and thalidomide have all been tried. Recently, some patients have demonstrated a good clinical response to either etanercept or anakinra. We report a case of a 10-year-old girl who experienced prolonged and severe inflammatory attacks, when she was treated with etanercept, and later with anakinra.

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References

  1. Drenth JPH, Cuisset L, Grateau G, Vasseur C, van de Velde-Visser SD, de Jong JG, Beckmann JS, van der Meer JWM, Delpech M (1999) Mutations in the gene encoding mevalonate kinase cause hyper-IgD and periodic fever syndrome. Nat Genet 22:178–181

    Article  PubMed  CAS  Google Scholar 

  2. Dreneth JPH, Haagsma CJ, van der Meer JW (1994) Hyperimmunoglobulinaemia D and periodic fever syndrome. The clinical spectrum in a series of 50 patients. International hyper-IgD study group. Medicine (Baltimore) 73:133–144

    Google Scholar 

  3. van der Hilst JC, Bodar EJ, Barron KS, Fenkel J, Drenth JPH, van der Meer JWM, Simon A et al (2008) Long-term follow-up, clinical features, and quality of life in a series of 103 patients with hyperimmunoglobulin D syndrome. Medicine (Baltimore) 87:301–310

    Article  Google Scholar 

  4. Lachmann HJ, Goodman HJ, Andrews PA, Gallagher H, Marsh J, Breuer S, Rowczenio DM, Bybee A, Hawkins PN (2006) AA amyloidosis complicating hyperimmunoglobulinaemia D with periodic fever syndrome: a report of two cases. Arthritis Rheum 54:2010–2014

    Article  PubMed  CAS  Google Scholar 

  5. Prietsch V, Mayatepek E, Krastel H, Haas D, Zundel D, Waterham HR, Wanders RJ, Gibson KM, Hoffman GF (2003) Mevalonate kinase deficiency: enlarging the clinical and biochemical spectrum. Paediatrics 111:258–261

    Article  Google Scholar 

  6. de Dios Garcia-Diaz J, Alvarez-Blanco MJ (2001) Glucocorticoids but not NSAID abort attacks in hyper-IgD, periodic fever syndrome. J Rheumatol 28:925–926

    PubMed  Google Scholar 

  7. Ostuni PA, Lazzarin P, Ongaro G, Gusi R, Todesco S, Gambari PF (1988) Hyper IGD syndrome: a new case treated with colchicine. Clin Rheumatol 7:398–401

    Article  PubMed  CAS  Google Scholar 

  8. Simon A, Drewe E, van der Meer JW, Powell RJ, Kelley RI, Stalenhoef AF, Drenth JP (2004) Simvastatin treatment for inflammatory attacks of hyperimmunoglobulinaemia D and periodic fever syndrome. Clin Pharmacol Ther 75:476–483

    Article  PubMed  CAS  Google Scholar 

  9. Topaloglu R, Ayaz NA, Waterham HR, Yüce A, Gumruk F, Sandal Ö (2008) Hyperimmunoglobulinaemia D and periodic fever syndrome: treatment with etanercept and follow-up. Clin Rheumatol 27:1317–1320

    Article  PubMed  Google Scholar 

  10. Drenth JP, Vonk AG, Simon A, Powell R, van der Meer JW (2001) Limited efficacy of thalidomide in the treatment of febrile attacks of the hyper-IgD and periodic fever syndrome: a randomized, double-blind, placebo-controlled trial. J Pharmacol Exp Ther 298:1221–1226

    PubMed  CAS  Google Scholar 

  11. Takada K, Aksentijevich I, Mahadevan V, Dean JA, Kelley RI, Kastner DL (2003) Favorable preliminary experience with etanercept in two patients with the hyperimmunoglobulinaemia D and periodic fever syndrome. Arthritis Rheum 48:2645–2651

    Article  PubMed  CAS  Google Scholar 

  12. Demirkaya E, Caglar MK, Waterham HR, Topaloglu R, Ozen S (2006) A patient with hyper IgD syndrome responding to anti-TNF treatment. Clin Rheumatol 26:1757–1759

    Article  PubMed  Google Scholar 

  13. Cailliez M, Garaix F, Rousset-Rouviere C, Bruno D, Kone-Paut I, Sarles J, Chabrol B, Tsimaratos M (2006) Anakinra is safe and effective in controlling hyperimmunoglobulinaemia D syndrome-associated febrile crisis. J Inherit Metab Dis 29:763

    Article  PubMed  CAS  Google Scholar 

  14. Bodar EJ, van der Hilst JCH, Drenth JPH, van der Meer JWM, Simon A (2005) Effect of etanercept and anakinra on inflammatory attacks in the hyper-IgD syndrome: introducing a vaccine provocation model. Neth J Med 63:260–264

    PubMed  CAS  Google Scholar 

  15. Rigante D, Ansuini V, Bertoni B, Pugliese AL, Avallone L, Federico G, Stabile A (2006) Treatment with anakinra in hyperimmunoglobulinaemiaD/periodic fever syndrome. Rheumatol Int 27:97–100

    Article  PubMed  Google Scholar 

  16. Drenth JP, van Deuren M, van der Ven-Jongerkrijg J, Schalkwijk CG, van der Meer JW (1995) Cytokine activation during attacks of hyperimmunoglobulinemia D and periodic fever syndrome. Blood 85:3586–3593

    PubMed  CAS  Google Scholar 

  17. Marchetti F, Barbi E, Tommasini A, Oretti C, Ventura A (2004) Inefficacy of etanercept in a chid with hyper-IgD syndrome and periodic fever. Clin Exp Rheumatol 22:791–792

    PubMed  CAS  Google Scholar 

  18. Nevyjel M, Pontillo A, Calligaris L, Tommasini A, D’Osualdo A, Waterham HR, Granzotto M, Crovella S, Barbi E, Ventura A (2007) Diagnostics and therapeutic insights in a severe case of mevalonate kinase deficiency. Paediatrics 119:e523–e557

    Article  Google Scholar 

  19. Lequerre T, Vittecoq O, Pouplin S, Klemmer N, Mejjad O, Daragon A, Prieur AM, Le Loet X (2007) Mevalonate kinase deficiency with structural damage responsive to anakinra. Rheumatology 46:1860–1862

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Royal Victoria Hospital, Belfast, Northern Ireland, UK

    Hiba M. Shendi, Deirdre Walsh & J. David M. Edgar

  2. Causeway Hospital, Coleraine, Northern Ireland, UK

    Deirdre Walsh

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  1. Hiba M. Shendi
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  2. Deirdre Walsh
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  3. J. David M. Edgar
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Correspondence to Hiba M. Shendi.

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Shendi, H.M., Walsh, D. & Edgar, J.D.M. Etanercept and anakinra can prolong febrile episodes in patients with hyperimmunoglobulin D and periodic fever syndrome. Rheumatol Int 32, 249–251 (2012). https://doi.org/10.1007/s00296-009-1322-8

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  • DOI: https://doi.org/10.1007/s00296-009-1322-8

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