Rheumatology Advance Access originally published online on November 8, 2005
Rheumatology 2006 45(1):120-121; doi:10.1093/rheumatology/kei146
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LETTER TO THE EDITOR |
Devic's syndrome in systemic lupus erythematosus and probable antiphospholipid syndrome
Social Hygiene Service, Department of Health, Hong Kong SAR; and 1 Department of Ophthalmology and Visual Sciences, Prince of Wales Hospital, Shatin, Hong Kong, People's Republic of China
Correspondence to: D. T. L. Liu, Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong. E-mail: david_tlliu{at}yahoo.com
SIR, Dr Ferreira and colleagues [1] have given an excellent description of a lupus patient with Devic's syndrome (neuromyelitis optica, NMO) but it seems that a fundamental issue of this case has not been answered. Was this patient suffering from NMO with inflammatory demyelinating disease targeting the optic nerves and spinal cord as described, or a genuine case of multiple sclerosis (MS) instead?
It is of paramount importance to make a clear distinction between these two entities; this is of interest not only academically but also because of the markedly disparate clinical outcomes and therapeutic strategies [2–4]. In the largest retrospective comparative study of 30 NMO and 50 MS patients, it has been shown that NMO usually carries a much graver prognosis [2]. Furthermore, NMO and MS differ substantially in treatment, as the immunomodulatory drugs typically used for MS will be ineffective for relapse prevention in NMO and severe refractory attacks in NMO usually necessitate rescue plasmapheresis or more effective immunosuppressive therapies [3].
In the management of this suspected case of NMO, the authors made use of investigatory tools such as cerebrospinal fluid analysis and MRI of the brain/spinal cord in diagnosis. However, as indicated by de Seze et al. [2], even when all these investigatory armaments are summoned there is still the pitfall of misdiagnosis and the inability to distinguish the two. Recently, using the technique of dual immunostaining, Lennon et al. [4] have demonstrated that a serum autoantibody marker, NMO-IgG, is highly specific for neuromyelitis optica and can significantly curtail the diagnostic ambiguity.
Perhaps, with the aid of a specific autoantibody marker, the management of this patient might have been more streamlined.
The authors have declared no conflicts of interest.
References
- Ferreira S, Marques P, Carneiro E, D’Cruz D, Gama G. Devic's syndrome in systemic lupus erythematosus and probable antiphospholipid syndrome. Rheumatology 2005;44:693–5.
[Free Full Text] - de Seze J, Lebrun C, Stojkovic T, Ferriby D, Chatel M, Vermersch P. Is Devic's neuromyelitis optica a separate disease? A comparative study with multiple sclerosis. Mult Scler 2003;9:521–5.
[Abstract/Free Full Text] - Wingerchuk DM, Weinshenker BG. Neuromyelitis optica. Curr Treat Options Neurol 2005;7:173–82.[Medline]
- Lennon VA, Wingerchuk DM, Kryzer TJ et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 2004;364:2106–12.[CrossRef][Web of Science][Medline]
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  L. R Mehta, M. K Samuelsson, A. K Kleiner, A. D Goodman, J. H Anolik, R J. Looney, and S. R Schwid Neuromyelitis optica spectrum disorder in a patient with systemic lupus erythematosus and anti-phospholipid antibody syndrome Multiple Sclerosis, April 1, 2008; 14(3): 425 - 427. [Abstract] [PDF]
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