ADPKD: Features

Key points

• ADPKD — important features: bilateral renal cysts with progressive kidney enlargement, hypertension, haematuria, nephrolithiasis and CKD; extrarenal cysts and vascular manifestations may occur.
• Recurrent UTIs can occur; cyst infection should be considered when there is systemic illness, focal pain and persistent fever.
• Abdominal/flank pain may result from cyst enlargement, haemorrhage, infection or stones.
• Chronic kidney disease develops progressively in many patients; rate of progression varies with genotype and other risk markers.
• Liver cysts are the commonest extrarenal manifestation and may cause hepatomegaly; intracranial aneurysms occur in selected patients/families.
• MRCP discriminator: family history + bilateral enlarged cystic kidneys + hypertension/CKD ± liver cysts → ADPKD.
• High-yield extrarenal clue: liver cysts; intracranial aneurysm screening is selective rather than universal.