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Autoimmun Rev. 2017 Jul;16(7):750-755. doi: 10.1016/j.autrev.2017.05.011. Epub 2017 May 5.

Peripheral nervous system involvement in systemic lupus erythematosus: Prevalence, clinical and immunological characteristics, treatment and outcome of a large cohort from a single centre.

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Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Catalonia, Spain.
Primary Care, Sillería Health Center, Toledo, Spain.
Department of Neurology, IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Catalonia, Spain.
Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Catalonia, Spain. Electronic address:


Disorders of peripheral nervous system in patients with systemic lupus erythematosus (PNS-SLE) are a major cause of morbidity. The aims of the present study were to determine the prevalence of PNS-SLE involvement in a large cohort of SLE patients from a single centre, to characterize such involvement, treatment modalities and outcome, and to identify the possible variables that may be associated with its presence. We performed an observational cross-sectional study that included all SLE patients being followed in our department between March and December 2015 who met at least one of the PNS-SLE case definitions proposed in 1999 by the American College of Rheumatology. Overall, 93 out of 524 (17,7%) patients presented with PNS-SLE syndrome; 90 (96.8%) of them were women with a mean age at PNS-SLE syndrome diagnosis was 44.8±14.1years and the average time from diagnosis of SLE to PNS-SLE diagnosis was 88 (range, 541-400) months. The most frequent manifestation was polyneuropathy (36.6%), followed by non-compression mononeuropathy (23.7%), cranial neuropathy and myasthenia gravis (7.5%, each), and Guillain-Barré syndrome (1.1%). The most frequent electrodiagnostic tests (EDX) pattern was axonal degeneration, present in 49 patients that corresponded to 80.3% of the overall EDX patterns. Mixed sensory-motor neuropathy was the most common type of involvement accounted for 56% of cases. Thirty-six out of 90 (40%) received glucocorticoids and/or immunosuppressant agents. Overall, global response (complete and/or partial) to treatments was achieved in 77.4% of patients without differences between the types of PNS-SLE involvement. Older age at SLE diagnosis (37.3±14.8 versus 30.8±12; p=0.001) and absence of hematologic involvement as cumulative SLE manifestation (11.8% versus 21.5%; p=0.034) had independent statistical significant associations with PNS-SLE development. The PNS-SLE involvement is not uncommon. Its most frequent manifestation is sensory-motor axonal polyneuropathy. The involvement occurs more frequently in patients who are diagnosed with SLE at older age. Prospective studies are needed to establish the incidence of PNS-SLE syndromes and the role of hematological manifestations in their development.


Electrodiagnostic tests; Neuropsychiatric systemic lupus erythematosus; Peripheral neuropathy; Systemic lupus erythematosus

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