Extended report
Transverse myelopathy in systemic lupus erythematosus: an
analysis of 14 cases and review of the literature
Birgit Kovacsa, Thomas L Laffertyb, Lawrence H Brenta, Raphael J DeHoratiusb
a Department of
Medicine, Albert Einstein Medical Center, Philadelphia, USA, b Department of Medicine,
Thomas Jefferson University, Philadelphia, USA
Correspondence to: Dr Brent, Einstein Arthritis Center, Albert Einstein Medical Center, 5501 Old York Road, Korman 103, Philadelphia, PA 19141, USA
Accepted for publication 13 October 1999
OBJECTIVE
To give a
comprehensive review of transverse myelopathy (TM), a rare but serious
condition reported in 1-2% of patients with systemic lupus
erythematosus (SLE).
METHODS
14 patients
with SLE and TM were evaluated and 91 additional cases published in the
English and German literature reviewed.
RESULTS
TM presented
either as the initial manifestation or within five years of the
diagnosis of SLE. Most patients presented with a detectable sensory
deficit at the thoracic level. In our 14 patients, 22% of the patients
showed complete neurological recovery, whereas in the total patient
population of 105 (our cases plus those reviewed in the literature),
complete recovery was observed in 50%, partial recovery in 29% and no
improvement or deterioration in 21%. Treatment with intravenous
methylprednisolone followed by cyclophosphamide seemed to be most
effective. Seventy per cent of the total patient population had
abnormal magnetic resonance imaging findings. In our group of 14 patients, those with higher disease activity (measured by the SLAM) at
onset of TM were treated more aggressively (for example, with
plasmapheresis and intravenous pulse cyclophosphamide). TM in our
patients was associated with antiphospholipid antibodies in 43% of the
cases as compared with 64% of the total patient population. Optic
neuritis occurred in 48% of the total patient population with SLE and
TM, suggesting an association.
CONCLUSIONS
TM in SLE
is a poorly understood entity. Outcome might be more favourable than
previously suggested. There is an association of TM with
antiphospholipid antibodies in SLE patients. Treatment including
intravenous cyclophosphamide may improve the final outcome. This report
emphasises the need for multicentre trials to establish guidelines for
optimal treatment.
© 2000 by Annals of the Rheumatic Diseases
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