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Abstract: TH-PO665

Guillain-Barre Syndrome Presenting Alongside Lupus Nephritis: Case and Management

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Mehta, Puja, Yale School of Medicine, New Haven, Connecticut, United States
  • Luciano, Randy L., Yale School of Medicine, New Haven, Connecticut, United States
  • Aklilu, Abinet Mathias, Yale School of Medicine, New Haven, Connecticut, United States
  • Malvar, Grace Landayan, Yale School of Medicine, New Haven, Connecticut, United States
Introduction

Systemic Lupus erythematosus (SLE) can affect any organ system including the nervous system. However, peripheral nervous system involvement occurs in less than 10% of cases. We present here a case of Guillain-Barre syndrome (GBS) coexisting in a patient with undiagnosed new onset lupus nephritis.

Case Description

The patient is a 21-year-old female with past medical history of well controlled skin-limited SLE treated with hydroxychloroquine who presented with two weeks of ascending bilateral muscle weakness and the inability to ambulate in the setting of a recent gastrointestinal infection. Due to high suspicion for Guillain-Barre syndrome (GBS), she was started on plasmapheresis (PLEX) and pulse dose steroids. Despite completing 5 sessions of PLEX, her condition worsened and required mechanical ventilation due to respiratory muscle involvement. She further developed locked-in-syndrome. She, then completed 5 days of IVIG without any improvement. On day 13, she was noted to have acute kidney injury (AKI) with nephrotic range proteinuria, along with rare dysmorphic RBCs on urine sediment. Serology was positive for an SLE flare including hypocomplementemia, elevated ANA titer, and a positive dsDNA antibody. A kidney biopsy demonstrated full house immunofluorescence, with electron microscopy revealing subepithelial deposits and mesangial dense deposits, consistent with lupus nephritis, class V. Despite treatment with steroids, she quickly developed non-oliguric AKI, requiring hemodialysis. She was started on mycophenolate mofetil along with prednisone, resulting in recovery of kidney function within one month of initiating immunosuppression. However, she had much slower recovery in GBS symptoms, resolving 6 months after initial presentation.

Discussion

It is difficult to speculate which came first: GBS or lupus nephritis, since we know GBS can be initial presentation for lupus nephritis, however, GBS can trigger lupus nephritis as well through aberrant immune activation. However, when GBS coexists with lupus nephritis, its treatment is controversial, with standard treatment (PLEX +/- IVIG) being less effective. Currently no specific guidelines exist to treat GBS in such situations, however, cyclophosphamide with corticosteroids is considered first line of treatment, with mycophenolate mofetil, as in this case, an alternative treatment option.