Guillain-Barre syndrome as the initial presentation of systemic lupus erythematosus: Case report with a systematic and literature review.

Systemic lupus erythematosus (SLE) can affect multiple systems in which central nervous system (CNS) involvement is common, but peripheral nervous system involvement is also increasingly being recognized. Guillian-Barre syndrome (GBS) as the first manifestation of SLE has been reported, but rare and not well understood. A 39-year female presented with GBS-like illness but on evaluation found to have features of SLE. Cerebrospinal fluid (CSF) showed characteristic albuminocytological dissociation and nerve conduction study (NCS) was suggestive of demyelinating polyradiculoneuropathy. On evaluation, she was found to have polyarthralgia, autoimmune hemolytic anemia, class I Lupus nephritis, mild splenomegaly, and pleural effusion. Serum antinuclear antibody was 4+ positive (coarse speckled) and extractable nuclear antigen profile revealed anti-dsDNA and anti-Sm antibody positivity, with low complement level. She fulfilled the diagnostic criterion of SLE and was managed with both plasmapheresis and pulse steroids followed by cyclophosphamide monthly pulse and oral hydroxychloroquine maintenance and showed significant improvement. The literature review showed only 26 cases reported till now. GBS without any obvious trigger should be extensively evaluated, as the underlying etiology will affect the treatment protocol as well as the prognosis. Our report highlights the significance of early recognition of SLE as a trigger of GBS, which changes conventional GBS treatment.