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PubMed This is a summary of 16 peer-reviewed journal articles Updated
Nephrology

Predicting the Future: Risk and Progression Scores

At a Glance

ADPKD progression is tracked using the Mayo Imaging Classification (kidney size and age) and the PROPKD score (genetics and medical history). These tools help determine if you are a rapid or slow progressor, which guides early treatment decisions and monitoring frequency.

One of the most common questions after an ADPKD diagnosis is: “What does my future look like?” Because the disease progresses differently for everyone, doctors use specialized tools called risk stratification scores to predict how your kidney function might change over time [1][2]. These scores help your care team identify “rapid progressors”—those who may benefit from earlier or more intensive treatment—versus “slow progressors” [3][4].

It is incredibly important to understand that kidney size typically increases for years or even decades before kidney function begins to decline [5][6]. This means your kidney function test (eGFR) might look perfectly normal, even though your kidneys are growing and the disease is silently progressing. This is why tracking kidney size is crucial.

The Mayo Imaging Classification (MIC)

The Mayo Imaging Classification is the most widely used tool for predicting ADPKD progression. It uses your height-adjusted total kidney volume (HtTKV) and your age to place you into one of five categories (1A through 1E) [2][7]. This system is based on the fact that larger kidneys for your age typically grow faster and lead to a quicker decline in kidney function [5][6].

  • Classes 1A and 1B (Slow Progressors): These patients typically have very slow cyst growth and are likely to maintain good kidney function for many decades [3][8].
  • Classes 1C, 1D, and 1E (Rapid Progressors): These categories represent kidneys that are growing more quickly. Patients in these groups typically see a faster decline in their eGFR and are often the primary candidates for disease-modifying treatments [3][4][9].

The PROPKD Score: A Clinical and Genetic View

While the Mayo Classification looks at your kidneys’ physical size, the PROPKD score looks at your genetic blueprint and medical history [1][10]. This score assigns points based on several factors:

Factor Points
PKD1 Truncating Mutation (The most severe genetic type) 4 points
PKD1 Non-Truncating Mutation 2 points
PKD2 Mutation 0 points
Male Sex 1 point
High Blood Pressure (Diagnosed before age 35) 2 points
Urological Event (First kidney stone, cyst infection, or blood in urine before age 35) 2 points

Source: [1][10]

Your total points (0–9) place you into a risk group: Low Risk (0–3 points), Intermediate Risk (4–6 points), or High Risk (7–9 points) [1][10]. This helps your doctor estimate the median age at which you might reach End-Stage Renal Disease (ESRD) [1].

Why These Scores Matter for Your Care

These tools are not meant to give you a “deadline,” but rather to act as a roadmap for your treatment [4].

  1. Treatment Decisions: If your scores show you are a rapid progressor, your doctor may recommend starting medications like tolvaptan, which has been shown to slow kidney growth and preserve function in high-risk patients [11][12].
  2. Monitoring Frequency: Slow progressors may only need imaging every few years, while rapid progressors may need more frequent blood work and blood pressure checks [3][13].
  3. Clinical Trials: Many research studies and clinical trials use these scores to find participants who are most likely to benefit from new experimental therapies [14][13].

By understanding your “class” and “score,” you can have a more nuanced conversation with your nephrologist about what to expect and how to protect your kidneys for the long term [15][16].

Common questions in this guide

What is the Mayo Imaging Classification for ADPKD?
The Mayo Imaging Classification is a tool that uses your age and height-adjusted total kidney volume (HtTKV) to predict how quickly your kidney disease will progress. It places patients into classes 1A through 1E to identify slow versus rapid progressors.
Why does my doctor measure my kidney size if my kidney function is normal?
In ADPKD, kidneys typically grow in size for years or decades before your kidney function (eGFR) begins to decline. Tracking kidney size helps predict future decline so you can start preventive treatments earlier.
What is the PROPKD score?
The PROPKD score evaluates your risk of disease progression based on your specific genetic mutation and clinical history. It assigns points for factors like having a PKD1 mutation, male sex, and developing high blood pressure or urological issues before age 35.
What does it mean if I am a rapid progressor?
Being a rapid progressor means your kidneys are growing more quickly and you may experience a faster decline in kidney function. Patients in this category are often primary candidates for disease-modifying treatments like tolvaptan to help slow down the disease.
Does a high risk score mean my kidneys will fail soon?
These scores are not meant to give you a strict deadline, but rather to act as a roadmap for your treatment. A higher score indicates an increased risk for earlier end-stage renal disease, which highlights the importance of proactive monitoring and early intervention.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is my exact Mayo Imaging Classification (1A-1E), and how was it calculated using my height and kidney volume?
  2. 2.Does my PROPKD score suggest that I am in the high-risk or intermediate-risk category?
  3. 3.Based on these scores, do you consider me a 'rapid progressor'?
  4. 4.How do these scores change my eligibility for treatments like tolvaptan?
  5. 5.If I am in a 'slow progressor' category (1A or 1B), how does that change the frequency of my monitoring and imaging?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (16)
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    The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease.

    Cornec-Le Gall E, Audrézet MP, Rousseau A, et al.

    Journal of the American Society of Nephrology : JASN 2016; (27(3)):942-51 doi:10.1681/ASN.2015010016.

    PMID: 26150605
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    Predicting Future Renal Function Decline in Patients with Autosomal Dominant Polycystic Kidney Disease Using Mayo Clinic Classification.

    Borrego Utiel FJ, Esteban de la Rosa RJ, Merino García E, et al.

    American journal of nephrology 2021; (52(8)):630-641 doi:10.1159/000518255.

    PMID: 34518464
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    Mayo imaging classification is a good predictor of rapid progress among Korean patients with autosomal dominant polycystic kidney disease: results from the KNOW-CKD study.

    Park HC, Hong Y, Yeon JH, et al.

    Kidney research and clinical practice 2022; (41(4)):432-441 doi:10.23876/j.krcp.21.261.

    PMID: 35286789
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    JAMA 2025; (333(19)):1708-1719 doi:10.1001/jama.2025.0310.

    PMID: 40126492
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    Hemorrhagic Cysts and Other MR Biomarkers for Predicting Renal Dysfunction Progression in Autosomal Dominant Polycystic Kidney Disease.

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    Journal of magnetic resonance imaging : JMRI 2021; (53(2)):564-576 doi:10.1002/jmri.27360.

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    Burden of Proof for Tolvaptan in ADPKD: Did REPRISE Provide the Answer?

    Mustafa RA, Yu ASL

    Clinical journal of the American Society of Nephrology : CJASN 2018; (13(7)):1107-1109 doi:10.2215/CJN.00190118.

    PMID: 29653956
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    Age- and height-adjusted total kidney volume growth rate in autosomal dominant polycystic kidney diseases.

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    Clinical and experimental nephrology 2019; (23(1)):100-111 doi:10.1007/s10157-018-1617-8.

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    Validation of the Mayo Imaging Classification System for Predicting Kidney Outcomes in ADPKD.

    Bais T, Geertsema P, Knol MGE, et al.

    Clinical journal of the American Society of Nephrology : CJASN 2024; (19(5)):591-601 doi:10.2215/CJN.0000000000000427.

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    Autosomal Dominant Polycystic Kidney Disease: Role of Imaging in Diagnosis and Management.

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    Can we further enrich autosomal dominant polycystic kidney disease clinical trials for rapidly progressive patients? Application of the PROPKD score in the TEMPO trial.

    Cornec-Le Gall E, Blais JD, Irazabal MV, et al.

    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2018; (33(4)):645-652 doi:10.1093/ndt/gfx188.

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    Clinical Utility and Tolerability of Tolvaptan in the Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD).

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    Drug, healthcare and patient safety 2022; (14()):147-159 doi:10.2147/DHPS.S338050.

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    Raynaud's phenomenon triggered by the vasopressin V2 receptor antagonist tolvaptan in a patient with autosomal dominant polycystic kidney disease and Sjögren's syndrome.

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    Prognostic Enrichment Design in Clinical Trials for Autosomal Dominant Polycystic Kidney Disease: The TEMPO 3:4 Clinical Trial.

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    Prognostic enrichment design in clinical trials for autosomal dominant polycystic kidney disease: the HALT-PKD clinical trial.

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    Insights into Autosomal Dominant Polycystic Kidney Disease from Genetic Studies.

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    Many lessons still to learn about autosomal dominant polycystic kidney disease.

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This page explains ADPKD progression scores for educational purposes only and does not constitute medical advice. Discuss your specific Mayo Classification and PROPKD score with your nephrologist to determine your personalized treatment plan.

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