Therapeutic plasma exchange in a patient with acute motor axonal neuropathy subtype of Guillain-Barre syndrome and systemic lupus erythematosus.

Abstract

A young female in her early 20s with a history of systemic lupus erythematosus presented to the emergency department due to 4 days of progressive bilateral extremity weakness and numbness. The patient reported flu-like symptoms that had spontaneously recovered 2 weeks prior to her presentation. She was 10 weeks pregnant at presentation. Lumbar puncture study and electrical muscle stimulation (EMS) were consistent with acute motor axonal neuropathy subtype of Guillain-Barre syndrome (GBS). Patient also had increased proteinuria and renal biopsy performed that was consistent with lupus nephritis. Despite treatment with pulse dose corticosteroids and IVIG, the patient had minimal neurological improvement and with continued decline required intubation. Her pregnancy was terminated at this point and a course of therapeutic plasma exchange (TPE) was started. Patient was also treated with cyclophosphamide. The patient responded to the combination of therapy and had slow but gradual neurologic recovery as well as improvement of proteinuria. Here we describe a case of an acute motor axonal neuropathy (AMAN) subtype of GBS in a young woman with active SLE and current pregnancy at the time of the presentation. Concurrent GBS and active SLE in the setting of pregnancy may be more treatment resistant, and combination therapy including TPE, immunosuppression, and termination of pregnancy may be indicated.