Rheumatology Advance Access originally published online on November 8, 2005
Rheumatology 2006 45(1):121; doi:10.1093/rheumatology/kei147
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LETTER TO THE EDITOR |
Devic's syndrome in systemic lupus erythematosus and probable antiphospholipid syndrome: reply
Departments of Internal Medicine and 1 Neuroradiology, São João Hospital, Porto, Portugal and 2 Lupus Research Unit, Guy's and St Thomas Hospital, London, UK
Correspondence to: S. Ferreira, Serviço de Medicina B, Hospital de São João, Alameda Hernâni Monteiro, 4200 Porto, Portugal. E-mail: susanaferreira14{at}hotmail.com
We thank Drs Chan and Liu for their interest in our case report. We entirely agree that it is fundamental to distinguish between neuromyelitis optica caused by an inflammatory/demyelinating process and a genuine case of multiple sclerosis. In fact, this was our main concern.
Our patient had clear evidence of lupus with multisystem involvement and had several clinical and laboratory signs of severe lupus activity, despite being strongly immunosuppressed. Her clinical presentation was not characteristic of multiple sclerosis: optic neuritis was unilateral; blindness was permanent; evoked potentials were delayed but also had short amplitude; and MRI lesions had no enhancement after gadolinium administration and were not progressive in time and space.
In our opinion, the main diagnostic issue was the differentiation between an inflammatory and/or a thrombotic mechanism. Antiphospholipid syndrome is common in lupus and is frequently associated with central nervous system involvement, namely transverse myelitis [1–3]. Also, abrupt-onset, unilateral and irreversible optic neuritis is more commonly ischaemic than inflammatory/demyelinating [1]. Our patient had transiently positive anticardiolipin antibody and presented with stroke, thrombocytopenia and extensive livedo reticularis, suggesting underlying antiphospholipid syndrome. A good response to anticoagulation has been reported in similar cases [2, 4].
Overall, we felt that a demyelinating syndrome as part of her lupus with secondary antiphospholipid syndrome was more probable than classical multiple sclerosis occurring coincidentally in this patient. We agree with Drs Chan and Liu that it would be interesting to study the specific autoantibody marker for neuromyelitis optica (NMO-IgG) [5]—this may well give further help in characterizing these clinically difficult patients.
The authors have declared no conflicts of interest.
References
- Ferreira S, D’Cruz D, Hughes G. Multiple sclerosis, neuropsychiatric lupus and antiphospholipid syndrome: where do we stand? Rheumatology 2005;44:434–42.
[Abstract/Free Full Text] - D’Cruz D, Mellor-Pita S, Joven B et al. Transverse myelitis as the first manifestation of systemic lupus erythematosus or lupus-like disease: good functional outcome and relevance of antiphospholipid antibodies. J Rheumatol 2004;31:280–5.[ISI][Medline]
- Lavalle C, Pizarro S, Drenkard C, Sánchez-Guerrero J, Alarcón-Segovia D. Transverse myelitis: a manifestation of systemic lupus erythematosus strongly associated with antiphospholipid antibodies. J Rheumatol 1990;17:34–7.[ISI][Medline]
- Aziz A, Conway MD, Robertson HJ, Espinosa LR, Wilson WA. Acute optic neuropathy and transverse myelitis in patients with antiphospholipid syndrome: favourable outcome after treatment with anticoagulants and glucocorticoids. Lupus 2000;9:307–10.
[Abstract/Free Full Text] - Lennon V, Wingerchuk D, Kryzer T et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 2004;364:2106–12.[CrossRef][ISI][Medline]
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