Background Acute transverse myelitis (ATM) is a severe complication of systemic lupus erythematosus (SLE). The prompt diagnosis and aggressive treatment should be followed because it often results in severe neurological sequelae. However, a little has been established the factors related with prognosis of ATM in SLE patients due to great rarity.
Objectives The aim of this study is to evaluate the clinical manifestations and factors related with outcome in patients with SLE-associated ATM.
Methods The retrospective analysis included 16 patients who were diagnosed with SLE-associated ATM at a single tertiary hospital from January 1995 to June 2014. The clinical characteristics, laboratory indices including cerebrospinal fluid (CSF), and radiologic image of the spine were obtained from the electronic medical records. The patients were divided into two groups based on improvement of neurologic deficits after treatments: 1) Non-responder and 2) Responder (completely or partially).
Results The median age at ATM onset was 33.5 years (IQR; 26.5-48.75) and 14 (87.5%) patients were female. The average follow-up duration after ATM onset was 74 months (range: 6-185 months). ATM was the first manifestation of SLE in 8 (50%). Parasthesia was found in 14 patients, motor weakness in 15, and autonomic dysfunction in 10. All patients started initially with high dose intravenous methylprednisolone (1mg/kg/day) therapy. And 14 (87.5%) and 9 (56.3%) were treated additionally with methylprednisolone pulse therapy (500mg/day–1000mg/day) and cyclophosphamide pulse therapy (500mg/m2). Consequentially, 12 (75%) patients recovered completely or partially (responder) and 4 (25%) persisted neurologic deficits (non-responder). Compared to responders, higher SLEDAI scores and lower complement levels were found in non-responders. Among 12 responders, five patients (36.4%) experienced at least one recurrence of ATM (range: 2-68 months after first episode). Compared to non-recurrent patients, CSF protein level was lower (36.2±11.2 vs. 122.7±89.5 mg/dL, p=0.032) and duration of high dose corticosteroid treatment was shorter (17.2±12.0 vs. 31.7±10.3 days, p=0.048) in the recurrent patients.
Conclusions Our results suggest that adequate intensity of steroid therapy should be considered initially in SLE-associated ATM to obtain favorable outcome.
Disclosure of Interest None declared