ADPKD Mayo Classification

2015Mayo Clinic

Mayo Imaging Classification — Autosomal Dominant Polycystic Kidney Disease

Classifies progression risk in ADPKD patients based on total kidney volume (TKV) and growth rate. Height-adjusted TKV (htTKV) is used to predict disease progression. High-risk patients may be candidates for clinical trials and tolvaptan therapy.

References

Irazabal MV, et al. Imaging Classification of Autosomal Dominant Polycystic Kidney Disease: A Simple Model for Selecting Patients for Clinical Trials. JASN 2015;26(1):160-172.
Chebib FT, Torres VF. Autosomal Dominant Polycystic Kidney Disease: Core Curriculum 2016. Am J Kidney Dis 2016;67(5):792-810.
Grantham JJ, et al. Volume Progression in Polycystic Kidney Disease. N Engl J Med 2006;354:2122-2130.

This classification system is intended for clinical decision support. Final diagnosis and treatment decisions rest with the clinician.

Categories

1AClass 1A — Low riskLow progression risk — slow GFR decline

Typical ADPKD, low htTKV (<150 mL/m)

htTKV <150 mL/m
Annual growth rate <1.5%
Bilateral cysts but kidney size relatively preserved
Usually in young patients (<30 years)

Management: Annual monitoring, blood pressure control, general protective measures

1BClass 1B — Low-intermediate riskLow-intermediate progression risk

htTKV 150-300 mL/m, moderately enlarged kidneys

htTKV 150-300 mL/m
Annual growth rate 1.5-3%
Bilateral multiple cysts, renal contour preserved

Management: Follow-up every 6-12 months, treatment adjustment, tolvaptan discussion

1CClass 1C — Intermediate riskIntermediate progression risk — ESRD likelihood increases within 10 years

htTKV 300-600 mL/m, significant kidney enlargement

htTKV 300-600 mL/m
Annual growth rate 3-4.5%
Kidneys significantly enlarged, corticomedullary differentiation lost

Management: Tolvaptan therapy should be considered, nephrology follow-up, GFR monitoring

1DClass 1D — High riskHigh progression risk — rapid GFR decline

htTKV 600-1500 mL/m, advanced kidney enlargement

htTKV 600-1500 mL/m
Annual growth rate >4.5%
Kidneys severely enlarged, multiple complicated cysts

Management: Tolvaptan therapy recommended, transplant evaluation, cyst complication management

1EClass 1E — Very high riskVery high progression risk — early ESRD

htTKV >1500 mL/m, massive kidney enlargement

htTKV >1500 mL/m
Annual growth rate >6%
Massive bilateral kidney enlargement, abdominal mass effect
Usually advanced age or truncating PKD1 mutation

Management: Tolvaptan therapy + transplant planning, nephrectomy discussion (symptomatic), dialysis preparation

References

Irazabal MV, et al. Imaging Classification of Autosomal Dominant Polycystic Kidney Disease: A Simple Model for Selecting Patients for Clinical Trials. JASN 2015;26(1):160-172.

Chebib FT, Torres VF. Autosomal Dominant Polycystic Kidney Disease: Core Curriculum 2016. Am J Kidney Dis 2016;67(5):792-810.

Grantham JJ, et al. Volume Progression in Polycystic Kidney Disease. N Engl J Med 2006;354:2122-2130.