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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.

Author information

1
Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Catalonia, Spain.
2
Primary Care, Sillería Health Center, Toledo, Spain.
3
Department of Neurology, IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Catalonia, Spain.
4
Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Catalonia, Spain. Electronic address: gespino@clinic.ub.es.

Abstract

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.

KEYWORDS:

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

PMID:
28483540
DOI:
10.1016/j.autrev.2017.05.011
[Indexed for MEDLINE]

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