Peripheral Nervous System Disease in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study

Abstract

Objective To determine the frequency, clinical characteristics, associations, and outcomes of different types of peripheral nervous system (PNS) disease in a multiethnic/multiracial, prospective inception cohort of systemic lupus erythematosus (SLE) patients. Methods Patients were evaluated annually for 19 neuropsychiatric (NP) events including 7 types of PNS disease. SLE disease activity, organ damage, autoantibodies, and patient and physician assessment of outcome were measured. Time to event and linear regressions were used as appropriate. Results Of 1,827 SLE patients, 88.8% were female, and 48.8% were white. The mean ± SD age was 35.1 ± 13.3 years, disease duration at enrollment was 5.6 ± 4.2 months, and follow‐up was 7.6 ± 4.6 years. There were 161 PNS events in 139 (7.6%) of 1,827 patients. The predominant events were peripheral neuropathy (66 of 161 [41.0%]), mononeuropathy (44 of 161 [27.3%]), and cranial neuropathy (39 of 161 [24.2%]), and the majority were attributed to SLE. Multivariate Cox regressions suggested longer time to resolution in patients with a history of neuropathy, older age at SLE diagnosis, higher SLE Disease Activity Index 2000 scores, and for peripheral neuropathy versus other neuropathies. Neuropathy was associated with significantly lower Short Form 36 (SF‐36) physical and mental component summary scores versus no NP events. According to physician assessment, the majority of neuropathies resolved or improved over time, which was associated with improvements in SF‐36 summary scores for peripheral neuropathy and mononeuropathy. Conclusion PNS disease is an important component of total NPSLE and has a significant negative impact on health‐related quality of life. The outcome is favorable for most patients, but our findings indicate that several factors are associated with longer time to resolution.

Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the ‘Attikon’ cohort

“…Compared to other large cohort studies, neuropsychiatric disease in our cohort does not seem to have significant differences in terms of risk factors, attribution rates, incidence, epidemiology and timing of NPSLE appearance. 11,19,[30][31][32][33][34] In our cohort, 15.1% of lupus subjects developed at least one primary neuropsychiatric manifestation, consistent with findings in other cohorts. 11,30 Only 14 (2.5%) patients developed 'minor' neuropsychiatric manifestations that were considered as attributed to SLE based on the presence of risk factors (e.g.…”

Neuropsychiatric events in systemic lupus erythematosus: a longitudinal analysis of outcomes in an international inception cohort using a multistate model approach

“…The NP events attributed to SLE varied from 17.9% (attribution model A) to 31.0% (attribution model B) and occurred in 13.5% (model A) to 21.2% (model B) of patients. Summary outcomes are provided in online supplementary tables S2 and S3 and detailed outcomes of individual manifestations may be found in previous publications 13 14 19–22…”

“…All NP events attributed to SLE using model A were included in the NP events using model B. All other events were classified as a non-SLE NP event 13 14…”

The management of neuropsychiatric lupus in the 21st century: still so many unmet needs?

“…Patients with PNS involvement were older at SLE diagnosis and had higher SLEDAI-2K scores. Although impairing health-related quality of life (HRQoL), the majority of neuropathies resolved or improved over time and cranial neuropathies had the steepest trajectory to resolution [ 13 ].…”