Childhood-onset primary Sjögren's Syndrome presenting as nephrotic syndrome: a case report and literature review.

Abstract

Background: Pediatric primary Sjögren's syndrome typically presents with oral and ocular dryness, along with a broad spectrum of extraglandular manifestations affecting multiple organ systems. Among renal manifestations, tubulointerstitial nephritis is most commonly observed, whereas glomerular involvement is exceedingly rare.

Case presentation: We report the case of an 8-year-old girl referred for evaluation of persistent foamy urine. Laboratory investigations revealed significant proteinuria and hypoalbuminemia. Kidney biopsy confirmed membranous nephropathy. Further evaluation indicated ocular involvement, evidenced by positive Schirmer's I test and reduced tear film break-up time. A labial salivary gland biopsy demonstrated focal lymphocytic infiltration. The patient was diagnosed with primary Sjögren's syndrome and was treated with corticosteroids and immunosuppressive agents, resulting in a favorable outcome and remission of proteinuria.

Conclusions: This case underscores the diverse clinical spectrum of primary Sjögren's syndrome and highlights the potential for rare glomerular involvement in children. It emphasizes the need for heightened awareness among pediatric healthcare providers regarding the systemic manifestations of primary Sjögren's syndrome to prevent delayed diagnosis.