Cystic kidney diseases: comparison table
- Topic
Cysts in the kidney range from harmless simple cysts to inherited diseases that end in kidney failure; one table sorts them.
What it is
Renal cysts are a frequent finding and a frequent exam topic. Inherited ciliopathies (ADPKD, ARPKD, nephronophthisis) and developmental disorders (multicystic dysplasia) must be separated from acquired cysts of dialysis and the simple cysts of aging. The age at presentation, kidney size, which part of the nephron forms the cysts and the organs outside the kidney give the answer.
Renal cystic diseases
| Disease | Inheritance | Presentation | Kidneys | Outcome |
|---|---|---|---|---|
| ADPKD | Autosomal dominant (PKD1, PKD2) | Adults; pain, hematuria, hypertension | Very large, bilateral cysts | Kidney failure in many by late middle age |
| ARPKD | Autosomal recessive (PKHD1) | Perinatal or childhood | Large, spongy, radial cysts | Neonatal death or childhood failure; liver fibrosis |
| Medullary sponge kidney | Mostly sporadic | Adults; stones, infection, hematuria | Normal size, cysts in medullary collecting ducts | Usually good |
| Nephronophthisis | Autosomal recessive (NPHP genes) | Childhood polyuria, growth failure | Small, cysts at corticomedullary junction | Kidney failure in youth |
| Multicystic dysplasia | Sporadic | Newborn flank mass | Usually one side, disorganized, cartilage | Other kidney carries the load |
| Acquired cystic disease | Acquired (dialysis) | Long-term dialysis | Small kidneys with many cysts | Higher risk of renal cell carcinoma |
| Simple cysts | Acquired, aging | Incidental | Single or few cortical cysts | Harmless |
Clinical notes
- Ultrasound criteria by age and family history diagnose ADPKD in families at risk.
- A complex cyst (septa, solid parts) needs evaluation for renal cell carcinoma.
Test yourself
Think of the answer, then tap the question to see it.
What cell structure is defective in most hereditary cystic kidney diseases?
The primary cilium of tubular epithelial cells (ciliopathies).
Which cystic disease is usually unilateral and contains cartilage?
Multicystic renal dysplasia.
Which cystic disease raises the risk of renal cell carcinoma in dialysis patients?
Acquired cystic kidney disease.
Elsewhere in DurgiPedia
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Polycystic kidney disease: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2025); Autosomal Dominant Polycystic Kidney Disease (StatPearls (NCBI Bookshelf), 2024).
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Representative model, for educational purposes only.