Focal segmental glomerulosclerosis in primary sjogren syndrome

Sjogren syndrome is an autoimmune disorder primarily affecting adults. Presentation in childhood is uncommon. Kidney manifestations chiefly include hypokalemia and renal tubular acidosis. The common pathological lesions which have been reported are tubulointerstitial nephritis, membranoproliferative glomerulonephritis, membranous nephropathy, and rarely, focal segmental glomerulosclerosis (FSGS). We report a patient of primary Sjogren syndrome, who presented with rough dry skin, recurrent parotid swellings, positive anti-Sjogren-syndrome-related antigen A autoantibodies, secondary nephrotic syndrome and acute kidney injury, with FSGS on histopathology, and remission of proteinuria following immunosuppression.