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

OBJECTIVE: To determine stroke prevalence, mechanisms, and long-term outcome in a cohort of Hispanic patients with systemic lupus erythematosus (SLE). METHODS: We analyzed demographical data, the timing between SLE diagnosis and stroke onset, stroke type, recurrence, and outcomes from an institutional database of 4451 patients with SLE followed from 1993 to 2018. RESULTS: We observed 139 strokes (3.1%), for an incidence rate of 1.25 per 1000 person-years: 81 (58.3%) acute ischemic stroke (AIS), 19 (13.7%) subarachnoid hemorrhage (SAH), 17 (12.2%) cerebral venous thrombosis, 13 (9.4%) intracerebral hemorrhage (ICH), and 9 (6.5%) transient ischemic attack. Median time from SLE diagnosis to acute stroke was 60 months (interquartile range 12-132 months). AIS had a bimodal presentation with 26% occurring within the first year and 30% >10 years after SLE diagnosis. In contrast, 75% of ICH cases occurred >3 years (and 34% >10 years) after SLE diagnosis. The most important cause of AIS was secondary antiphospholipid syndrome (48%). Hypertension was associated with 69% of ICH cases, while aneurysmal rupture was observed in 78% of SAH cases. Excellent recovery at hospital discharge was observed in 65%. Stroke recurrence was observed in 7%. The long-term all-cause fatality rate was 8%. CONCLUSIONS: The prevalence of stroke in this cohort was 3.1%. Ischemic strokes had a bimodal presentation, occurring either early after SLE diagnosis or after a several-year delay. Half of the hemorrhagic strokes occurred >10 years after the diagnosis of SLE. Clinical outcome was usually good with a relatively low recurrence rate.

Intrathecal formation of anticardiolipin antibodies in a patient with SLE-related relapsing longitudinal myelitis: a possible pathogenic connection.

BACKGROUND: Nontraumatic acute transverse myelitis (ATM) can occur in response to infectious, inflammatory and vascular triggers; 1% of patients with systemic lupus erythematosus (SLE) develop ATM, but the mechanism remains unknown. OBJECTIVE: The objective of this case report is to describe a case of intrathecal formation of anticardiolipin antibodies (aCL) during SLE-related ATM. METHODS: A single patient analysis was conducted. RESULTS: A 26-year-old housewife was diagnosed with SLE at age 19. Circulating aCL antibodies were positive at diagnosis. At age 21, she developed an episode of severe sepsis. At 23 years of age she developed an episode of ATM that left her paraplegic with a D10 sensory level, from which she recovered partially. Three years later, she developed a clinical relapse of ATM. During that second episode, serum levels of aCL were within normal limits, while cerebrospinal fluid levels were increased, suggesting intrathecal production of aCL. CONCLUSION: Here, we present a case of a woman who developed relapsing SLE-related longitudinally extensive ATM in whom intrathecal formation of aCL was demonstrated, suggesting that local production and cross-recognition of nervous tissue by those autoantibodies may be myelopathic.

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.

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.