To present a series of seven cases of acute onset fulminant peripheral neuropathy (PN), mimicking Guillain-Barré syndrome (GBS), secondary to active systemic lupus erythematosus (SLE) and identify important clinical factors that differentiate these patients from typical GBS patients without SLE. 

GBS is a rare complication of SLE, and previous case reports have suggested that important clinical and prognostic differences exist between these patients and those with typical GBS without SLE. 

Retrospective, observational data was collected from seven patients who presented with fulminant PN secondary to SLE between 1999-2022. All patients met the American College of Rheumatology (ACR) diagnostic criteria for SLE, and all patients were diagnosed with GBS by Neuromuscular Medicine staff.   

There were 7 patients (71% female) with GBS secondary to active SLE identified with a mean age of onset of 32 years (range 14-51). There were no preceding symptoms or prior diagnosis of SLE before onset of neuropathy symptoms in 3/7 of cases. The mean time of neuropathy onset to nadir was 2.4 months (range 0.5-6). Nerve conduction study and electromyography (EMG) was completed in all but one patient, and findings were suggestive of either diffuse motor-predominant axonal polyradiculoneuropathy (2/6) or mixed sensorimotor axonal polyradiculoneuropathy (4/6). There was minimal to no symptomatic response following initial first-line therapies in 5/7 cases. Cyclophosphamide and high dose steroids were utilized in 2/7 and 6/7 cases, respectively. Following the acute management phase, all 7 patients remained on chronic immunosuppression regimens that included combinations of low dose steroid, hydroxychloroquine, mycophenolate mofetil, rituximab, and azathioprine.

Cases of GBS secondary to SLE pose a significant challenge to neurologists as they often have no preceding symptoms or diagnosis of SLE, may show no demyelinating features on EMG, have minimal to no response to first-line therapies, and may have protracted courses requiring chronic immunosuppression.