Myelitis in systemic lupus erythematosus: clinical characteristics and effect in accrual damage. A single-center experience

Abstract

Objective The purpose of this study was to describe the clinical characteristics of acute transverse myelitis, including the time of their presentation, and to evaluate their effect on accrual damage in patients with systemic lupus erythematosus (SLE). Methods Patients with SLE who were hospitalized because of incident, noninfectious myelitis at our institute between January 1997 and December 2013 were identified. As a control group, we selected for each of the patients in the study group one SLE patient hospitalized at the closest date to the case due to other severe non-neuropsychiatric (NP) SLE manifestation, with no history of NP manifestations or noninfectious disease. Clinical characteristics, laboratory results, treatment, disease activity (SLEDAI-2K), and damage (SLICC/ACR-DI) were collected from medical charts at the index hospitalization and one year after hospitalization. Results Demographics and SLE characteristics, including age at SLE diagnosis and time since SLE diagnosis to hospitalization, were comparable in patients with myelitis and controls. At hospitalization, disease activity and cumulative damage were similar in both groups. Patients with myelitis received more aggressive treatment than controls. One year after hospitalization, two of the 15 patients who completed follow-up had symptom improvement without neurologic sequelae, and 13 of them had some improvement of symptoms with neurologic sequelae. Four patients died in the myelitis group, three of them of infectious diseases, and one of alveolar hemorrhage. No patient died because of myelopathy and in the control group no patient died, although three were lost during the follow-up. Disease activity and treatment did not differ between both groups. However, cumulative damage was higher among the patients with myelitis than controls (1.9 ± 0.9 vs 0.75 ± 0.9; p = 0.003). Conclusion Patients with myelitis have clinical characteristics similar to those observed in non-NP SLE and receive more aggressive treatment. Furthermore, myelitis is associated with a significant increase in accrual damage compared with severe non-NP manifestations.

Aquaporin-4-IgG positive neuromyelitis optica spectrum disorder and systemic autoimmune diseases overlap syndrome: a single-center experience

“…Our group previously reported that myelitis in SLE has a significant effect in accrual damage measured with SLICC/ACR-DI. 21 In the present study, we also found a statistically significant difference when excluding neurological items attributable to NMOSD from the SLICC/ACR-DI scoring in SLE/NMOSD patients, implying that NMOSD has an important effect on accrual damage in SLE patients. We did not see the same effect on pSS as SSDDI excludes damage not associated directly to pSS.…”

“…19 Furthermore, according to the 2015 IPND criteria the presence of SLE, pSS or myasthenia gravis in AQP4-IgG seropositive NMOSD patients strengthens the confidence about an NMOSD diagnosis. 1 Both SLE and pSS may present with demyelinating central nervous system manifestations in the form of myelitis and optic neuritis in similar patterns than NMOSD, [20][21][22] and consequently, some of these cases with classic NMOSD clinical syndromes were considered neuropsychiatric manifestations of the underlying disorder before the advent of AQP4-IgG antibody testing. Overall, AQP4-IgG antibodies have been detected in 0.6-3% and 10% of SLE and pSS cohorts, respectively.…”

Natural history of longitudinally extensive transverse myelitis in 35 Hispanic patients with systemic lupus erythematosus: good short-term functional outcome and paradoxical increase in long-term mortality

“…19 Shortterm outcome from 19 of the subjects included in the present report had been reported previously. 20,21 Antiphospholipid syndrome (APS): Throughout follow-up, we used three different defining criteria for APS; [22][23][24] those criteria were not mutually exclusive and we always used the most current one. However, for the present manuscript we considered APS according to the Sydney criteria.…”

Spinal cord syndromes in patients with systemic lupus erythematosus: differentiating lupus myelitis, neuromyelitis optica, and multiple sclerosis

“…4 Several case series and small case-control studies have examined patients with SLE myelitis and have found that the clinical presentation, laboratory evaluation, and radiographic features of this disease are often heterogeneous. 5–20 In addition, several other autoimmune conditions may affect the spinal cord. Among them, multiple sclerosis (MS) and anti-aquaporin-4 antibody (AQP4)-mediated neuromyelitis optica (NMO) may be difficult to distinguish clinically from SLE myelitis.…”