Pediatric systemic lupus erythematosus presenting with protein-losing enteropathy: A rare diagnostic challenge
Main Article Content
Abstract
Protein-losing enteropathy (PLE) is a rare gastrointestinal manifestation of systemic lupus erythematosus (SLE). A 12-year-old girl presented for treatment with generalized edema, pleural effusion, and ascites due to persistent severe hypoalbuminemia, with markedly elevated stool alpha-1 antitrypsin (> 236.25 mg/dL). She had alopecia, high ESR (> 96 mm/h), hypercoagulability, and refractory dyslipidemia. Laboratory findings showed low C3/C4, positive ANA (1:100, speckled), mildly elevated anti-dsDNA, and negative anti-Sm, SSA, SSB, anticardiolipin, anti-β2GPI, and lupus anticoagulant. Small bowel and colonic biopsies revealed mild deposition of IgG, IgM, C3c, and C1q in capillary and small vessel endothelium. The patient was treated with IVIG (1 g/kg/day) and methylprednisolone (30 mg/kg/day) for 3 days plus mycophenolate mofetil, resulting in resolution of edema, normalization of albumin, and improvement of hypercoagulability. This case suggests that PLE may be an initial manifestation of pediatric SLE and responds well to timely immunosuppression.
Article Details
Keywords
Protein-losing enteropathy (PLE), Systemic lupus erythematosus (SLE), Lupus-associated protein-losing enteropathy (LUPLE), children
References
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