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

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.

journal_name

Autoimmun Rev

journal_title

Autoimmunity reviews

authors

Toledano P,Orueta R,Rodríguez-Pintó I,Valls-Solé J,Cervera R,Espinosa G

doi

10.1016/j.autrev.2017.05.011

subject

Has Abstract

pub_date

2017-07-01 00:00:00

pages

750-755

issue

eissn

1568-9972

issn

1873-0183

pii

S1568-9972(17)30133-7

journal_volume

pub_type

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