Isolated end-stage renal disease in Sjögren's syndrome due to immune complex mediated glomerulonephritis

Renal involvement is an uncommon extra-glandular manifestation in Sjogren's syndrome (SS). We present the case of a young male who presented with nephrotic range proteinuria and advanced irreversible renal disease, with positive anti-Ro antibody (Ab) and antineutrophil cytoplasmic antibody (ANCA) with myeloperoxidase (MPO) specificity. He was initially treated with steroids for suspected ANCA vasculitis but treatment was discontinued as there was no response and renal biopsy revealed interstitial lymphocytic infiltrates, advanced glomerular disease with immune complex mediated glomerulonephritis more suspicious for SS. SS usually affects exocrine glands and patients rarely have renal involvement in which cases it is commonly tubulo-interstitial nephritis. This report describes a rare case of anti-Ro Ab and MPO-ANCA positive patient with advanced irreversible renal disease due to immune-complex mediated glomerulonephritis felt to be due to SS and without any classic sicca symptoms.