Massive proteinuria and autosomal dominant polycystic kidney disease: a rare coincidence.
Autosomal dominant polycystic kidney disease (ADPKD) with nephrotic syndrome is a rare coincidence. Among 19 reported cases since 1972, focal glomerulosclerosis is the dominant reported pathology. Here, we report the 6th case of focal segmental glomerulosclerosis with ADPKD. A 29-year-old man with a history of APCDK presented with massive proteinuria. He had a history of concurrent leptospirosis and brucellosis, and trace proteinuria and mild hypertension had been diagnosed 4 years earlier. Urine study showed proteinuria (21 g/d) and hematuria. Kidney biopsy report was compatible with focal and segmental sclerosis. The patient received prednisolone and cyclosporine. After 4 months, proteinuria decreased to 600 mg/d. Patients with ADPKD who show massive proteinuria should undergo kidney biopsy. It is possible that different mutations in these patients could clarify the nature of this coincidence.
Department of Nephrology, Firoozgar General Hospital, Tehran University of Medical Sciences, Tehran, Iran. ssavaj@hotmail.com
Shokoufeh
S
Savaj
Mahmoud
M
Parvin
Javad
J
Savoj
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications* / Q000473:pathology / Q000652:urine; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications*; D011239:Prednisolone / Q000627:therapeutic use; D011507:Proteinuria / Q000209:etiology*
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone