Guillain-barre ́ syndrome as the initial manifestation of pediatric systemic lupus erythematosus

Background: Various neurological manifestations of pediatric systemic lupus erythematosus (pSLE) have been reported. However, Guillain-Barré syndrome (GBS) as the initial presentation has rarely been documented. We report a case of the acute motor axonal neuropathy (AMAN) subtype of GBS as the first presentation of pSLE.
Case presentation: A previously healthy 11-year-old girl was admitted for a viral upper respiratory tract infection. Subsequently, she developed acute progressive weakness and respiratory failure. Nerve conduction velocity findings were compatible with the AMAN subtype of GBS. Further investigations revealed proteinuria, hematuria, elevation of Antinuclear antibody(ANA) levels, anti-dsDNA, and a decrease in complement components. Pediatric SLE was diagnosed using the The Systemic Lupus Collaborating Clinics (SLICC) criteria. The GBS did not improve after intravenous immunoglobulin therapy, plasmapheresis (PE), or pulse methylprednisolonebut gradually improved after the third cycle of intravenous cyclophosphamide, with complete recovery at 4 months after the treatment.
Conclusion: To our knowledge, this is the first and youngest case of pSLE in which the patient presented with GBS and achieved complete recovery following immunosuppressive therapy. Our findings suggest intravenous pulses of cyclophosphamide to be the most effective treatment in the case of GBS and SLE.