Peripheral nervous system involvement accounts for fewer than 10% of SLE cases with neuropsychiatric manifestations. Guillain-Barré syndrome (GBS) as the presenting, major manifestation of pediatric SLE is extremely rare, and the best treatment approach is unknown. A 14-year-old, previously healthy female teenager developed classic features of GBS with ascending bilateral muscle weakness leading to respiratory insufficiency, associated with protein-cell dissociation in cerebro-spinal fluid, nerve root enhancement by MRI and reduction in compound muscle action potential amplitude. SLE was diagnosed serologically and histologically (lupus nephritis WHO class II). Despite immediate treatment with intravenous immunoglobulin (IVIg), methylprednisolone pulses and subsequently, rituximab, the patient required prolonged mechanical ventilation. She achieved full recovery following 14 PLEX treatments and two more rituximab infusions. Anti-dsDNA, C3, C4 and urinalysis normalized while anti-Smith and Sjögren antibodies persisted 15 months after disease onset, with no other lupus manifestations. Review of the literature revealed two pediatric cases of GBS at the onset of SLE and a third case with GBS 6 years after the diagnosis of SLE. Conventional GBS therapy may not be adequate to treat SLE-GBS. SLE should be included in the differential diagnosis of GBS. Importantly, treatment experiences and outcomes of such cases need be reported to inform future treatment recommendations.

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Background: Peripheral nervous system (PNS) involvement, including Guillain Barré Syndrome (GBS), accounts for fewer than 10% of SLE cases with neuropsychiatric manifestations. GBS as the presenting, major manifestation of pediatric SLE is extremely rare, and the best treatment approach is unknown. Case presentation: A 14-year-old, previously healthy female Emirati teenager presented with a classic picture of GBS with ascending, progressive bilateral muscle weakness leading to respiratory insufficiency within five weeks of symptom onset, associated with typical protein-cell dissociation in cerebro-spinal fluid and nerve root enhancement demonstrated by spinal MRI. Subsequently, elevated anti-dsDNA and anti-Smith/RNP and anti-SS-A and -B antibody concentrations were detected in serum, suggestive of SLE. GBS treatment was initiated with IVIG and methylprednisolone pulses, with minimal improvement. The patient required endotracheal intubation and ventilation, followed by a second course of IVIG, rituximab, and eventually plasma exchange (PLEX) therapy. The diagnosis of lupus-associated GBS was corroborated by a kidney biopsy demonstrating lupus nephritis WHO class II with “full house” immunofluorescence pattern. After 14 PLEX sessions, her muscle strength and respiratory efforts had improved substantially. Treatment was completed with two more rituximab infusions, followed by mycophenolate mofetil, in addition to HCQ and tapering doses of oral prednisolone. Five weeks after the last PLEX treatment, she had regained her usual strength and achieved full, sustained recovery from GBS. While she continued to demonstrate moderate anti-dsDNA antibodies and high-level anti-Smith and Sjogren antibodies, C3, C4 and urine readings quickly normalized with no other manifestations of lupus or lupus nephritis 11 months after the initial assessment. At this time she was maintained with hydroxychloroquine, with ongoing depletion of circulating B cells. To our knowledge, this is only the third pediatric patient reported with SLE-GBS.Conclusions: We report severe GBS as the first, dominant manifestation of pediatric SLE. Our case and a review of the literature reveal that cconventional GBS therapy may not be adequate to treat this rare lupus presentation. SLE should be included in the differential diagnosis of GBS. Importantly, treatment experiences and outcomes of such cases need be reported to inform future treatment recommendations.