The Diagnosis of Lupus Nephritis in A Patient with Autosomal Dominant Polycystic Kidney Disease: A Rare Case Report.

Autosomal dominant polycystic kidney disease (ADPKD), as a widespread inherited cystic kidney disease has a prevalence of ~1/1000 live births. However, there are rare reports of the association of ADPKD with nephrotic range proteinuria such as lupus nephritis (LN). In this study, we report a patient with ADPKD who manifested a sudden increase of urinary protein excretion with positive anti-double stranded DNA and antinuclear antibody tests. Finally, based on percutaneous ultrasound-guided renal biopsy LN was proved. This report advises clinicians to evaluate ADPKD patients periodically and perform complementary clinical and laboratory investigations in cases with unusual presentations such as nephrotic syndrome.