Therapeutic Plasma Exchange in a Patient With Acute Motor Axonal Neuropathy Subtype of Guillain-Barre Syndrome and Systemic Lupus Erythematosus

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Objective To highlight treatment resistance of concurrent Guillain-Barre syndrome (GBS) and active systemic lupus erythematosus (SLE). Background Coincidence of SLE and GBS is an uncommon and complicated course of autoimmune disease. Treatment is further complicated by pregnancy exacerbation of SLE. Design/Methods We report a case of acute motor axonal neuropathy (AMAN) subtype of GBS in a young pregnant woman in her early 20s presenting with SLE. Patient was 10 weeks pregnant at presentation. Lumbar puncture study and electrical muscle stimulation (EMS) were consistent with acute motor axonal neuropathy subtype of GBS. Patient also had increased proteinuria and renal biopsy performed that was consistent with lupus nephritis. Despite treatment with IVIG and pulse dose corticosteroids, the patient had minimal neurological improvement with respiratory decline requiring intubation. Her pregnancy was terminated at this point and a course of therapeutic plasma exchange (TPE) was started followed by cyclophosphamide. Results The patient responded to the combination of therapy and had slow but gradual neurologic recovery as well as improvement of proteinuria. Conclusions 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. For patients with concurrent GBS and SLE, especially axonal subtype of GBS and during pregnancy, TPE should be considered as a primary treatment option with respect to both efficacy and safety.