Guillain-Barré syndrome in the puerperium as the initial manifestation of systemic lupus erythematosus: a case report with severe axonal variant

Abstract

Case Presentation: A 24-year-old previously healthy female patient developed, in the immediate puerperium following a vaginal delivery complicated by fetal death, a condition characterized by ascending weakness, dyspnea, and respiratory failure, requiring invasive mechanical ventilation and tracheostomy. On neurological examination, she presented with flaccid tetraparesis, diffuse areflexia, and decreased tactile and pain sensation. Cerebrospinal fluid analysis showed albuminocytologic dissociation, and electroneuromyography demonstrated a predominantly axonal sensorimotor polyneuropathy, consistent with Guillain-Barré syndrome. During hospitalization, she developed persistent fever and elevated inflammatory markers, along with positive autoantibodies (antinuclear antibody up to 1:640 with a homogeneous pattern, positive anti-dsDNA and anti-Ro antibodies) and a positive direct Coombs test. She progressed with complicated puerperal endometritis, requiring hysterectomy. She was treated with intravenous immunoglobulin and pulse methylprednisolone therapy, with progressive clinical improvement and successful ventilatory weaning. She was discharged with residual proximal paresis, global hypotonia, and mild sensory deficit. During outpatient follow-up, she showed partial recovery of strength, especially in the upper limbs, with persistent significant deficits in the lower limbs and sphincter dysfunction. Laboratory evidence of autoimmune activity persisted, and azathioprine was initiated, resulting in improvement of systemic symptoms. Discussion: Guillain-Barré syndrome in the puerperium is a rare condition, possibly related to immune reconstitution during this period. The presence of specific autoantibodies, systemic manifestations, and a positive Coombs test strongly suggests an underlying systemic autoimmune disease, particularly systemic lupus erythematosus as a possible initial manifestation. Peripheral nervous system involvement in systemic lupus erythematosus is uncommon and may mimic acute inflammatory polyneuropathy. The axonal form, as observed in this case, is associated with greater clinical severity and slower recovery. Recognizing this association has important diagnostic and therapeutic implications. Final Comments: This case highlights the importance of investigating autoimmune etiologies in patients with Guillain-Barré syndrome in atypical contexts, such as in the puerperium. Early identification of this association may directly impact management and prognosis, reinforcing the need for a multidisciplinary approach.