Myelitis in systemic lupus erythematosus: clinical features, immunological profile and magnetic resonance imaging of five cases

系统性红斑狼疮合并脊髓炎:5例病例的临床特征、免疫学特征及磁共振成像表现

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Abstract

Myelopathy is one of the neuropsychiatric lupus syndromes. In this article, an original series of related lupus myelitis is reported and analyzed. We employed a retrospective chart review and identified all patients who were admitted to a general hospital in Buenos Aires, Argentina, with SLE and myelitis during the period 2007-2014. Five patients were observed, all women. The mean age was 25.4 years (19-39). In three of five cases, myelitis was one of the initial SLE manifestations. The SLE Disease Activity Index was variable (3/5 with high activity). Time to nadir ranged from 6 to 72 h. All had severe impairment, with motor deficit, sensory level and urinary retention. Magnetic resonance imaging was abnormal in all cases, 3/5 presented a longitudinally extensive myelitis. Serum analysis revealed positive antinuclear antibodies at a high titer in all patients, 4/5 had low complement levels and 3/5 had anti-phospholipids positive. The treatment (methylprednisolone and, in some cases, cyclophosphamide, anticoagulation and/or plasmapheresis) produced partial improvement or no benefits. One patient died due to sepsis. The others showed significant disability at 6 months (European Database for Multiple Sclerosis grading scale=6-8). In view of these results, myelitis associated with lupus shows heterogeneity of the clinical, radiological and serological features. In our experience, the cases were severe and with poor response to treatment. Further studies are required to understand this disease and establish a more efficient treatment.

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