Background/Purpose: Whilst Neurological disorders are a common manifestation of systemic lupus erythematosus (SLE), peripheral neuropathies have received little attention. The literature consists mostly of small case series with very few studies investigating larger samples. Consequently, various features of SLE-associated peripheral neuropathies such as prevalence, symptoms, severity, chronicity, clinical and serological associations, and electrophysiological and biopsy findings, are poorly described. Small fibre neuropathies are almost entirely un-reported. The aim of this study is to determine the prevalence, clinical features, electrophysiological and biopsy findings of peripheral neuropathies in systemic lupus erythematosus and to identify clinical and laboratory correlations.

Methods: We performed a retrospective study of 2,097 SLE patients seen at a single Center since 1987, and ascertained for the presence of neuropathy according to diagnostic criteria laid down by the American Academy of Neurology.   We categorized subtypes of peripheral neuropathies on the basis of symptoms, examination, electrophysiological studies, and punch skin biopsy features. We excluded patients with co-morbid features associated with neuropathies.   We evaluated for an association of demographic, SLE-related clinical features, antibodies, and other immunological data in SLE patients with neuropathies versus without neuropathies by student’s t-test or chi-squared test, with p-values <0.05 considered statistically significant.

Results: The prevalence of neuropathies was 4.4%, present in 82 of 2097 patients.   Of these 82 patients, the most common neuropathy was a large fiber neuropathy (68.3%):  including neurophysiological assessment demonstrating 36 (44%) symmetric axonal neuropathies and 42 (51%) patients with a pure axonal loss, 18 patients had small-fiber neuropathies, characterized by decreased intra-epidermal nerve fiber density and/or morphological changes of unmyelinated fibers on skin biopsy.   Altogether, patients with neuropathies were older at time of SLE diagnosis (39.16 versus 32.05, p<0.0001), had an increased risk of Zoster infection, but otherwise had similar profile of SLE-related clinical and laboratory features).

Conclusion: Our characterization of small-fiber neuropathy in patients, has never been systematically evaluated and reported in unselected SLE cohorts, and suggests that this neuropathy may be an unrecognized cause of morbidity in SLE patients.   SLE patients with neuropathies versus without neuropathies shared similar clinical and immunological features.  Given the tropism of VZV for peripheral neuronal structures, prospective studies are warranted to evaluate whether re-activation of VZV is a risk factor for SLE neuropathies.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/peripheral-neuropathy-in-systemic-lupus-erythematosus/