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

Abstract

Background and objective Acute transverse myelitis (TM) is an infrequent neurological complication of systemic lupus erythematosus (SLE). Short-term outcome varies widely between cohorts. Little is known about the epidemiology and long-term functional outcome of TM associated to SLE. Methods Patients with SLE and acute TM were identified during hospital admission, visits to the Emergency Room or the Neurology Outpatient Clinic. We evaluated ambispectively those patients with SLE presenting with clinical myelopathy and corroborated with spinal MRI. Cases were divided as partial (non-paralyzing) or complete (paralyzing). We determined long-term functional outcome as well as mortality in those patients with follow-up periods of at least five years. Results We identified 35 patients (partial, n = 15; complete, n = 20) in which complete clinical and imaging data were available (26 with follow-up ≥ 5 years). Patients with complete TM were significantly older than those with partial forms. Positive antiphospholipid antibodies were observed in 80% of patients, suggesting a possible mechanistical role. Surprisingly, functional recovery at one year was in general good; however, we observed a five-year mortality of 31% because of sepsis (in 10 cases) or pulmonary embolism (in one case). Conclusions Short-term outcome of SLE-related TM is generally good, and recurrence rate is low. However, we observed a long-term fatality rate of 31% for reasons unrelated to TM, suggesting that TM is a manifestation of severe immune dysregulation and a predictor of severity and mortality in patients with SLE.

Stroke in systemic lupus erythematosus: epidemiology, mechanism, and long-term outcome

“…29,30 APS is also a well-established risk factor for stroke and other NPSLE syndromes. [31][32][33][34] On that note, a key finding of our study was that a high proportion of cases of cerebral ischemia was associated with secondary APS, supporting a mechanistic role. Although in our series, ischemic stroke was mainly attributed to a hypercoagulable state associated with APS, other concomitant mechanisms related to APS but independent of ischemia (e.g.…”

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

“…7 Overall we found a low prevalence of aPL antibodies in contrast to the high prevalence found in lupus myelitis of up to 80%. 32 It has long been suggested that these autoantibodies play a pathogenic role in lupus myelitis; furthermore, intrathecal formation of anticardiolipin antibodies have been reported. 33,34 Whether the presence of aPL plays a role in NMOSD coexisting with SLE/ pSS remains to be determined.…”

Neuropsychiatric Manifestations of Antiphospholipid Syndrome—A Narrative Review

“…Transverse myelitis in association with APS is rare, with an estimated prevalence of approximately 1% [ 12 , 19 ] presenting with symmetrical paraparesis, sensory loss and sphincter dysfunction. As with many of the neurological manifestations associated with APS, transverse myelitis is also seen in patients with SLE and that is where most of the literature is focused [ 120 , 121 ]. Case series have demonstrated a strong association between aPL and transverse myelitis in SLE, with a prevalence of aPL between 73% and 100% [ 122 , 123 ].…”