Abstract
Protein-losing enteropathy (PLE) is a rare but recognized manifestation of systemic lupus erythematosus (SLE). As an initial presentation of SLE, PLE is exceptionally uncommon, particularly in pediatric patients. We report the case of a 15-year-old Vietnamese girl with no significant past medical or family history, who presented with PLE as the initial manifestation of SLE. Clinical features included bilateral eyelid and lower extremity edema, ascites, and hypoalbuminemia, in the absence of nephrotic-range proteinuria, hepatic dysfunction, or malnutrition. Stool α1-antitrypsin concentration was markedly elevated at >231 mg/dL (normal <26.8 mg/dL), supporting the diagnosis of PLE in conjunction with clinical features and therapeutic response. Immunological evaluation revealed positive antinuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA) antibody, and lupus anticoagulant, hypocomplementemia, along with proteinuria equivalent to >0.5 g/24 h, fulfilling the 2019 EULAR/ACR classification criteria for SLE. The patient also developed cerebral venous sinus thrombosis. Treatment with corticosteroids, hydroxychloroquine, and warfarin resulted in significant clinical improvement. At 7 months of follow-up, she remained clinically stable, with normalized serum albumin levels and resolution of thrombosis. This case highlights the challenges of diagnosing PLE as an initial symptom of SLE in resource-limited settings. Heightened awareness of this rare presentation can facilitate early diagnosis and optimal management, improving patient outcomes.