Hyperimmunoglobulinemia D with recurrent fever

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: HIDS

Overview

Hyperimmunoglobulinemia D with recurrent fever (commonly abbreviated as HIDS) is a periodic fever syndrome originally described in 1984 by the internist Prof. Jos van der Meer, then at Leiden University Medical Centre. No more than 300 cases have been described worldwide.

Pathophysiology

Is it not known how mevalonate kinase mutations cause the febrile episodes, although it is presumed that other products of the cholesterol biosynthesis pathyway, the prenylation chains (geranylgeraniol and farnesol) might play a role.

Causes

Virtually all patients with the syndrome have mutations in the gene for mevalonate kinase, which is part of the HMG-CoA reductase pathway, an important cellular metabolic pathway (Drenth et al 1999, Houten et al 1999). Indeed, similar fever attacks (but normal IgD) have been described in patients with mevalonic aciduria - an inborn error of metabolism now seen as a severe form of HIDS.

Differentiating Hyperimmunoglobulinemia D with recurrent fever from other Diseases

The differential diagnosis includes fever of unknown origin, familial Mediterranean fever (FMF) and familial Hibernian fever (or TNFα reception associated periodic syndrome/TRAPS).

Epidemiology and Demographics

It has mainly been described in The Netherlands and France, although the international registry includes a number of cases from other countries.

Diagnosis

History and Symptoms

HIDS is one of a number of periodic fever syndromes. It is characterized by attacks of fever, arthralgia, skin lesions, and diarrhea.

Laboratory Findings

Laboratory features include an acute phase response (elevated CRP and ESR) and markedly elevated IgD (and often IgA), although cases with normal IgD have been described.

Medical Therapy

The recurring fevers are highly unpleasant for patients, but so far only the immunosuppressant drugs etanercept (Enbrel) and anakinra have been shown to be effective. Statin drugs might decrease the level of mevalonate and are presently being investigated.

References

  • Online 'Mendelian Inheritance in Man' (OMIM) 260920
  • van der Meer JWM, Vossen JM, Radl J, van Nieuwkoop JA, Meyer CJLM, Lobatto S, van Furth R. Hyperimmunoglobulinaemia D and periodic fever: a new syndrome. Lancet 1984;I:1087-1090. PMID 6144826.
  • Drenth JP, Cuisset L, Grateau G, Vasseur C, van de Velde-Visser SD, de Jong JG, Beckmann JS, van der Meer JW, Delpech M. Mutations in the gene encoding mevalonate kinase cause hyper-IgD and periodic fever syndrome. International Hyper-IgD Study Group. Nat Genet 1999;22:178-81. PMID 10369262.
  • Houten SM, Kuis W, Duran M, de Koning TJ, van Royen-Kerkhof A, Romeijn GJ, Frenkel J, Dorland L, de Barse MMJ, Huijbers WAR, Rijkers GT, Waterham HR, Wanders RJA, Poll-The BT. Mutations in MVK, encoding mevalonate kinase, cause hyperimmunoglobulinaemia D and periodic fever syndrome. Nature Genet 1999;22:175-177. PMID 10369261.
  • Rigante D, Ansuini V, Bertoni B, Pugliese AL, Avallone L, Federico G, Stabile A. Treatment with anakinra in the hyperimmunoglobulinemia D/periodic fever syndrome. Rheumatol Int. 2006 Jul 27. PMID 16871408

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