Peripheral nervous system involvement in systemic lupus erythematosus: a retrospective study on prevalence, associated factors and outcome

Abstract

In the past years, the peripheral nervous system involvement in systemic lupus erythematosus (PNSLE) has received little attention despite its potentially significant impact on disease outcome.Objectives. To assess the prevalence and clinical features of PNS involvement in a large cohort of Systemic Lupus Erythematosus (SLE) patients.Methods. SLE patients consecutively observed at two tertiary referral centres over a period of 14 years (from 2000 to 2014) were selected. PNS manifestations were ascertained according to the 1999 American College of Rheumatology case definitions and by using an attribution algorithm for neuropsychiatric (NP) events.Prevalence of PNSLE, demographic, clinical and laboratory data were assessed. Patients with PNS manifestations were compared with a control group of SLE patients without PNS involvement.Results. In a retrospective cohort of 1,224 patients, the overall prevalence of PNS involvement was 6.9% (85 patients, 95% Confidence Interval (95%CI) 0.06-0.08), with 68% of the events attributable to SLE. Polyneuropathy was the most common manifestation observed (42 events, 43.3%), followed by cranial neuropathy in 30 cases (30.9%), 12 singlesingle (12.4%) or multiple (8 events, 8.2%) mononeuritis. The average age of SLE onset was significantly higher in patients with PNS manifestations than in controls (mean ± standard deviation (SD): 45.9±14.8 vs 37.1±14.0) and they were more likely to have a higher SLEDAI-2K and SLICC/ACR Damage Index (SDI) scores, and hypertension and livedo reticularis. A subgroup analysis for events deemed to be SLE-related provided similar results.Conclusion. PNS involvement is an uncommon, but not so rare complication of SLE. A careful neurological evaluation for this manifestation should be included in the diagnostic workup, especially in patients with later onset and with higher damage and disease activity.

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

“…The present study supports and expands the findings of previous work . The overall frequency of PNS events in our study (7.6%) was higher than that reported by Oomatia et al (5.9%) but lower than that in the other 2 large cohort studies (14% and 17.7%) .…”

Livedo racemosa in neurological diseases: an update on the differential diagnoses

“…Systemic lupus erythematosus is a chronic immunemediated connective tissue disease affecting multiple organ systems and characterized by the generation of autoantibodies, deposition of immune complexes and activation of the complement system [25]. The antibodies most commonly associated with SLE include antinuclear antibodies, anti-double-stranded DNA and anti-Sj€ ogren's-syndrome-related antigen A antibodies [26].…”

Ganglionic Acetylcholine Receptor Antibodies and Autonomic Dysfunction in Autoimmune Rheumatic Diseases

“…* Mean abnormal value from autonomic function test data. 1 BP, blood pressure; 2 HR, heart rate; 3 CVRR, coefficient of variation in R-R intervals; 4 LF/HF, low frequency/high frequency; 5 MIBG, metaiodobenzylguanidine; 6 H/M ratio, heart-to-mediastinum ratio; 7 N.D., not done; 8 W.N.L., within normal limits. Anhidrosis was confirmed before immunotherapy, except on the axillary and palmar surfaces.…”

“…Autonomic neuropathy has been reported in autoimmune rheumatic diseases including Sjögren's syndrome (SS) [1,2], systemic sclerosis (SSc) [3], rheumatoid arthritis (RA) [4], and systemic lupus erythematosus (SLE) [5]. Several underlying mechanisms such as the immunological basis, which includes circulating autoantibodies, abnormalities of cellular immunity, vasculitis, and secondary amyloidosis have been proposed.…”