Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature

Objective-To give a comprehensive review of transverse myelopathy (TM), a r are 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 yea rs of the diagnosis of SLE. Most patients presented with a detectable senso ry deficit at the thoracic level. In our 14 patients, 22% of the patients s howed complete neurological recovery, whereas in the total patient populati on of 105 (our cases plus those reviewed in the literature), complete recov ery was observed in 50%, partial recovery in 29% and no improvement or dete rioration in 21%. Treatment with intravenous methylprednisolone followed by cyclophosphamide seemed to be most effective. Seventy per cent of the tota l patient population had abnormal magnetic resonance imaging findings. In o ur group of 14 patients, those with higher disease activity (measured by th e SLAM) at onset of TM were treated more aggressively (for example, with pl asmapheresis and intravenous pulse cyclophosphamide). TM in our patients wa s associated with antiphospholipid antibodies in 43% of the cases as compar ed 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 an tiphospholipid 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.