P2.210: Guillain-Barré Syndrome: A Rare Initial Manifestation of Systemic Lupus Erythematosus in a 15-Year-Old Female

Introduction: Juvenile-onset Systemic Lupus Erythematosus (SLE) is a complex multisystemic autoimmune disease that can affect the nervous system leading to neuropsychiatric manifestations. However, peripheral nervous systemic involvement is less frequent. We describe a rare presentation of Guillain-Barré Syndrome (GBS) as the initial manifestation of SLE.

Case Description: A 15-year-old girl with a history of migraines was admitted for new onset ascending paralysis that progressed over two weeks. She was non-ambulatory with absent reflexes in her bilateral lower extremities. MRI spine demonstrated diffuse enhancement of cauda equina nerve roots. Electromyography results were consistent with early axonal demyelination. Weakness continued despite intravenous immunoglobulin (IVIG) for suspected GBS, but additional treatment with corticosteroids and plasmapheresis resulted in additional improvement. She subsequently developed palatal ulcers, diffuse blistering bullous lesions, pericardial effusion, and hypertension. She had nephrotic range proteinuria, hypocomplementemia (C3= 38mg/dL, C4= 2mg/dL), positive ANA (1:5120), positive dsDNA, Smith, RNP, SSA, SSB, ribosomal P and cardiolipin IgG antibodies. Renal biopsy showed diffuse proliferative and early-stage membranous glomerulonephritis (ISN/RPS class IV and V lupus nephritis) with "full house" fluorescence positivity. Although laboratory studies were drawn after IVIG, the clinical presentation, hypocomplementemia, antibody profile and renal biopsy were conclusive for SLE. She developed acute superficial thrombophlebitis of the right cephalic vein requiring anticoagulation. Hydroxychloroquine, mycophenolate mofetil, rituximab and pulse glucocorticoids were administered, providing further improvement of her GBS. Belimumab (for renal involvement) and dapsone (for bullous lesions) were initiated with moderate improvement. She developed hypoxia and dyspnea, and HRCT chest showed necrotizing pneumonia, and was also concerning for SLE-related pulmonary hemorrhage, leading to transfer to an outside hospital. Bronchoscopy demonstrated pulmonary hemorrhage with 30% hemosiderin laden macrophages. She was started on monthly intravenous cyclophosphamide with overall improvement in her pulmonary, neurological, renal, and mucosal disease, leading to eventual transition to outpatient services. She was able to ambulate. Belimumab and dapsone were discontinued. She completed 6 doses of cyclophosphamide and was switched to mycophenolate mofetil. The oral steroids are being tapered.

Discussion: Although GBS-like acute axonal neuropathies is uncommon in patients with SLE, there is an association between the two conditions. In our patient, early recognition of the multisystemic symptoms and establishing the diagnosis of SLE was critical so that additional immunosuppressive agents could be initiated. In our patient, early initiation of glucocorticoids and immunosuppressive agents resulted in improvement. Additionally, this patient continues to require multidisciplinary collaboration between several pediatric subspecialists. This case underscores the importance of early recognition and treatment of SLE.

Conclusion: Autoimmune demyelinating polyneuropathy, GBS, is a rare but severe initial presentation of SLE. SLE should be considered as part of the differential diagnosis when first-line therapy for GBS fails.