Lupus Patient Develops Miller Fisher Variant of Guillain-Barré Syndrome

The etiology of GBS in SLE patients is complex and poorly understood, but it likely involves different immunological mechanisms. It’s not clear whether GBS unmasks an underlying autoimmune disorder such as SLE, or if a lupus flare triggers GBS. In our patient, it seems a lupus flare triggered her GBS manifestation. It’s possible both may occur concurrently—her episode occurred during the winter months, when viral syndromes are common. GBS is an immune mediated condition and cellular and humoral mechanisms are involved.¹

The three main pathophysiological mechanisms include the following.¹

1. GBS and SLE have a common trigger through molecular mimicry, which causes cross-reactivity because many GBS cases develop after infection. Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Zoster virus, Haemophilus influenza are known viral culprits. Vaccines have been indicated as well.⁶ Through this same mechanism, an immune response against neurons may contribute to SLE-induced neuropathy.
2. A polyneuropathy may develop after a widespread immunological response in SLE that may cause autoantibody formation against gangliosides, such as GQ1b.
3. A vascular complication of SLE, including microangiopathy, premature atherosclerosis, vasculitis and anti-phospholipid antibodies, can lead to ischemic demyelination, which may trigger a GBS-like response.

Despite similarities and differences, SLE and GBS present therapeutic challenges. Important: Treatment will differ when managing an SLE patient with GBS.

Although high-dose corticosteroid use is commonplace in SLE flares, it is not a typical first-line therapy for GBS. The main modalities of GBS therapy include plasma exchange (PLEX) and/or the administration of intravenous immunoglobulin (IVIG). These treatments hasten GBS recovery, as shown in randomized controlled trials.²

Patients recover sooner when treated early. The beneficial effects of plasma exchange and IVIG are believed to be equivalent, although combination of these treatments is not beneficial.³

Studies have shown glucocorticoids are not beneficial in GBS alone and do not alter the outcome of GBS, but could possibly help neuropathies. In a systematic review and meta-analysis of six trials with 587 participants, glucocorticoid-treated patients with GBS showed no significant difference in disability grade compared with patients not treated with glucocorticoids.⁴

In an updated 2012 meta-analysis of six randomized controlled trials and 649 patients with GBS, treatment with plasma exchange was superior to supportive care.⁵ When comparing IVIG with plasma exchange for GBS treatment, the study found IVIG was as effective as plasma exchange.⁴ In our patient, plasma exchange was chosen for therapy.

Summary

Multiple central and peripheral neurologic complications can occur in SLE. We have highlighted the rare neurologic manifestations of SLE associated with GBS. This process is largely thought to be stimulated by an immunological process such as molecular mimicry.