Autosomal Dominant Polycystic Kidney Disease
Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder that causes numerous kidney cysts, enlargement and gradual loss of function.
Key takeaways
- Disease progression varies widely even within families, so kidney function and imaging need individual interpretation.
- Blood-pressure control and avoidance of kidney injury are central regardless of whether a progression-slowing medicine is used.
- Flank pain, blood in urine and cyst infection have different causes and should not be treated as interchangeable complications.
The listings below are not suitable for every person with ADPKD; treatment depends on progression risk, liver health, kidney function and monitoring capacity.
Monitoring progression
Assessment may include estimated kidney filtration, urine testing, blood pressure and total kidney volume on imaging. Family history and genetic testing can clarify diagnosis in selected cases. Liver cysts are common, and some families have increased intracranial aneurysm risk that warrants targeted screening rather than universal imaging.
Treatment priorities
Manage blood pressure, cardiovascular risk, hydration advice and medicines that may harm kidneys. Tolvaptan may slow progression in selected adults at risk of rapid decline but causes substantial urination and requires regular liver monitoring. Pain, stones and infection need cause-specific treatment.
When to seek urgent care
Seek urgent care for sudden severe headache, fever with flank pain, inability to pass urine, heavy visible blood in urine, fainting or severe persistent abdominal or back pain.