Correspondence

Mevalonate Kinase Deficiency and Autoinflammation

N Engl J Med 2007; 357:1871-1872November 1, 2007

Article

To the Editor:

The Perspective article about mevalonate kinase deficiency and autoinflammatory disorders by Haas and Hoffmann (June 28 issue)1 accompanies the Brief Report by Neven et al.2 The Perspective describes the disease spectrum of human mevalonate kinase deficiency, which includes unexplained periodic episodes of fever and inflammation, and discusses treatment regimens used to alleviate symptoms in patients with the disease. In this context, we believe it is necessary to clarify statements in the Perspective that imply that a causal effect of the inflammation in patients with mevalonate kinase deficiency may relate to a type 2 helper T cell bias (increased levels of interleukins 4, 5, and 6) resulting in hyper-IgD, hyper-IgE, or both. This effect has not been reported in humans, and at this time, this observation pertains to data collected in studies of our and Hoffmann's murine model3 of mevalonate kinase deficiency that were not cited in the Perspective.

Elizabeth J. Hager, Ph.D.
K. Michael Gibson, Ph.D.
University of Pittsburgh School of Medicine, Pittsburgh, PA 15261
elizabeth.cothren@chp.edu

3 References

1. 1

   Haas D, Hoffmann G. Mevalonate kinase deficiency and autoinflammatory disorders. N Engl J Med 2007;356:2671-2673
   Full Text | Web of Science | Medline

2. 2

   Neven B, Valayannopoulos V, Quartier P, et al. Allogeneic bone marrow transplantation in mevalonic aciduria. N Engl J Med 2007;356:2700-2703
   Full Text | Web of Science | Medline

3. 3

   Gibson KM, Tse T, Pappu A, Steiner R, Hoffmann G, Hager E. Chronic inflammation and hyper-IgD/IgE in mice with targeted deletion of the mevalonate kinase gene. J Inherit Metab Dis 2007;30:Suppl 1:45-45
   Web of Science

Citing Articles (2)

Citing Articles

1. 1

   ANNALISA MARCUZZI, ALESSANDRA PONTILLO, LUIGINA DE LEO, ALBERTO TOMMASINI, GIULIANA DECORTI, TARCISIO NOT, ALESSANDRO VENTURA. (2008) Natural Isoprenoids are Able to Reduce Inflammation in a Mouse Model of Mevalonate Kinase Deficiency. Pediatric Research 64:2, 177-182
   CrossRef

2. 2

   (2008) Current awareness: Pharmacoepidemiology and drug safety. Pharmacoepidemiology and Drug Safety 17:5, i-xvi
   CrossRef