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Rheumatology Advance Access originally published online on June 14, 2005
Rheumatology 2006 45(3):360-361; doi:10.1093/rheumatology/keh715
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© The Author 2005. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org


LETTER TO THE EDITOR

Re: Aouba et al. Crowned dens syndrome misdiagnosed as polymyalgia rheumatica, giant cell arteritis, meningitis or spondylitis

S. Rajakulendran, D. Smith1 and C. Deighton

Departments of Rheumatology and 1 Radiology, Derbyshire Royal Infirmary, London Road, Derby DE7 6GW, UK

Correspondence to: C. Deighton. E-mail: chris.deighton{at}derbyhospitals.nhs.uk

SIR, we thank Aouba et al. for their interesting series of eight cases of crowned dens syndrome with atypical presentations [1]. We have concerns about some of their conclusions on the management of these patients. Their argument is that a variety of clinical presentations may be caused by acute pyrophosphate arthritis of the tissues surrounding the dens, and that if a CT scan identifies calcification in this area then this will reduce the need for invasive procedures such as temporal artery biopsies and lumbar punctures.

The difficulty in diagnosing pyrophosphate arthropathy on the basis of radiological calcification in any joint is that this may be an incidental finding, particularly in patients in their eighth or ninth decade [2, 3]. Consequently, to diagnose acute pyrophosphate arthritis confidently, crystals would need to be identified in inflamed synovial fluid. For acute crystal disease around the dens, this would be hazardous and impractical. Chondrocalcinosis may become less evident if crystals are shed from cartilage during acute or recurrent inflammatory episodes [4, 5]. Therefore, serial CT scans around the time of the acute episode may provide some reassurance of changing calcification coinciding with the period of inflammation, but would be impractical and involve considerable radiation.

Clearly, there are considerable difficulties in confidently stating that potentially sinister symptoms around the head and neck are unequivocally caused by crowned dens syndrome. We would conclude that a patient presenting with symptoms suggestive of temporal arteritis or meningitis needs to have these conditions thoroughly investigated, and have appropriate treatment instituted urgently whilst waiting for the results. This would apply whether they have a calcified dens on a CT scan or not. Under these clinical circumstances we feel that it is inappropriate to suggest that negative temporal artery biopsies and normal lumbar punctures are ‘invasive, expensive and useless investigations’ [1].

C.D. is an Associate Editor of Rheumatology but was not involved in the editorial process for this paper. The other authors have declared no conflicts of interest.

References

  1. Aouba A, Vuillemin-Bodaghi V, Mutschler C, De Bandt M. Crowned dens syndrome misdiagnosed as polymyalgia rheumatica, giant cell arteritis, meningitis or spondylitis: an analysis of eight cases. Rheumatology 2004;43:1508–12.[Abstract/Free Full Text]
  2. Mitrovic DR, Stankovic A, Iriarte-Borda O et al. The prevalences of chondrocalcinosis in the human knee joint. An autopsy survey. J Rheumatol 1988;15:633–41.[Medline]
  3. Felson DT, Anderson JJ, Naimark A et al. The prevalence of chondrocalcinosis in the elderly and its association with knee osteoarthritis: the Framingham study. J Rheumatol 1989;16:1241–5.[Web of Science][Medline]
  4. Doherty M, Dieppe PA, Watts I. Pyrophosphate arthropathy: a prospective study. Br J Rheumatol 1993;32:189–96.[Abstract/Free Full Text]
  5. Doherty M, Dieppe PA. Acute pseudogout: ‘crystal shedding’ or acute crystallisation? Arthritis Rheum 1981;24:954–7.[Medline]
Accepted 17 May 2005


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This Article
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