Unusual presentation of Evans syndrome with hypokalemic paralysis during pregnancy, management and outcome

Background: Evans Syndrome (ES) is a rare autoimmune disorder characterized by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). Its occurrence in association with Sjögren’s Syndrome (SS) during pregnancy, compounded by hypokalemic paralysis, is exceedingly rare.
Case Description: A 24-year-old primigravida at 17 weeks of gestation presented with limb weakness due to hypokalemic paralysis. She had a history of ES and SS diagnosed prior to conception, managed initially with corticosteroids and vitamin B12 supplementation. During pregnancy, she experienced hypokalemia, renal tubular acidosis, and moderate anemia, which were managed effectively with potassium, bicarbonate, prednisolone, and hydroxychloroquine. The pregnancy was monitored closely, culminating in a cesarean delivery of a healthy baby at 39 weeks. Postpartum care included transfusions and anticoagulation therapy. Both mother and baby showed favorable outcomes without major complications.
Conclusion: The coexistence of ES and SS during pregnancy, further complicated by hypokalemic paralysis, presents unique challenges. Early diagnosis, multidisciplinary management, and close monitoring are pivotal in achieving successful maternal and neonatal outcomes.