Guillain-Barré syndrome as first presentation of systemic lupus erythematosus: a rare manifestation complicated by IVIg-induced splenic infarct

Abstract

A 44-year-old woman presented with progressively worsening neurological symptoms of 1 week duration. Physical examination revealed absent reflexes of the lower extremities and proximal muscle weakness, bilaterally. Cerebrospinal fluid analysis and electrophysiological studies were consistent with the diagnosis of Guillain-Barré syndrome (GBS) and the patient was started on intravenous immunoglobulin infusion. Along with positive neurological findings, rheumatological work up revealed elevated antinuclear antibody titres, positive double-stranded DNA and anti-Smith antibodies. These results, in conjunction with positive clinical findings, confirmed an underlying diagnosis of systemic lupus erythematosus (SLE). The patient's hospital course was complicated by an episode of severe left upper quadrant abdominal pain, fever, tachycardia and elevated inflammatory markers. CT scan of the abdomen revealed a splenic infarct following completion of IVIg infusion, making this a contributor to thrombus formation in the setting of an already thrombophilic state, and a rare complication of an approved method of treatment.

Fulminant Guillain-Barré syndrome in a patient with systemic lupus erythematosus

“…Review of the literature reveals that cases of GBS in the context of SLE have predominantly presented with acute inflammatory demyelinating polyneuropathy followed by the acute motor axonal neuropathy variant 6–19. Rare cases of acute motor sensory axonal neuropathy,14 20 Miller-Fisher syndrome21 and pharyngeal-cervical-brachial syndrome22 have also been reported.…”

“…Rare cases of acute motor sensory axonal neuropathy,14 20 Miller-Fisher syndrome21 and pharyngeal-cervical-brachial syndrome22 have also been reported. While standard GBS therapies such as plasma exchange and IVIG are often applied, in the majority of cases of concurrent SLE and GBS, additional immunosuppressive or immunomodulatory therapies have been employed, including corticosteroids, cyclophosphamide, azathioprine,14 mycophenolate mofetil17 and/or hydroxychloroquine 13 14 16 18 22. In some cases, patients did not respond to traditional GBS therapies, but had significant clinical improvement following immunosuppressive therapy 10 14 23…”

The introspection on the diagnosis and treatment process of a case of Guillain–Barré syndrome (GBS) attributed to systemic lupus erythematosus (SLE)

“…[ 15 , 22 ] The exact pathophysiology of this relationship is largely unknown; however, cross-reactivity between the auto-antibodies associated with SLE and those formed against myelin tissue is believed to be involved. [ 26 ] In addition, immune-suppression associated with SLE is an aggravating factor which exacerbates the neuronal inflammation in these patients. [ 25 ] The older people were more likely to suffer from PNS-SLE than those without peripheral neuropathies.…”

Guillain–Barré syndrome occurring synchronously with systemic lupus erythematosus as initial manifestation treated successfully with low-dose cyclophosphamide

“…GBS as initial manifestation of lupus is exceedingly rare and has been reported in a few cases in the literature ( 8 – 12 ). We report a 38-year-old female who presented with AIDP as initial manifestation of SLE.…”