ADPKD Progression

Understanding progression

Video

Progression

Autosomal dominant polycystic kidney disease (ADPKD) continues to get worse over time

You might notice signs but not know they’re from ADPKD. As your cysts grow, your kidneys can't always keep up with the body’s demands. Kidney damage builds up. Even if kidney function looks normal. Eventually, the damage is too much, and the kidneys slowly stop working.

How do doctors measure kidney function?

Doctors use something called GFR, or glomerular filtration rate, to see how well your kidneys are working to filter your blood. The amount of creatinine—a waste product—you have in your blood is used to estimate your GFR. You've probably talked to your doctor about your creatinine levels. Doctors use a blood test to measure creatinine.

When creatinine levels go up, GFR goes down. The higher your GFR, the better your kidneys are working. Doctors use GFR to tell what stage of chronic kidney disease, or CKD, someone has.

One unit of GFR is equal to 1 mL/min/1.73m2.

≥90 Normal function Mild reduction Mild to moderate reduction Lower Higher 60-89 45-59 Moderate to severe reduction 30-44 Severe reduction 15-29 End-stage renal disease (ESRD) <15 GFR (mL/min/1.73m 2 ) 2 1 3a 3b 4 5 CKD stage Kidney function Creatinine
≥90 Normal function 60-89 Mild reduction 45-59 Mild to moderate reduction 30-44 Moderate to severe reduction 15-29 Severe reduction <15 End-stage renal disease (ESRD) GFR (mL/min/1.73m 2 ) and kidney function 1 2 3a 3b 4 5 CKD stage Creatinine Lower Higher

 

When doctors talk about “disease stages” in ADPKD, they are using these CKD stages.

Fast progression

People with fast-progressing or rapidly progressing ADPKD reach ESRD at a younger age than other patients with ADPKD

With fast-progressing ADPKD, kidney function gets worse and cysts grow more quickly, leading to earlier kidney failure. Larger kidneys can lead to earlier high blood pressure, more pain, earlier kidney damage, and early kidney failure.

Kidney size is one sign of risk for fast-progressing ADPKD

Doctors can measure your kidney volume based on imaging or scans, and will adjust based on your height. Then, they will see if they are big for your age.

For instance, 2 people might have the same size kidneys. But in a shorter person they would be a greater risk. Or, if 2 people of the same height have the same size kidneys, the younger person may be at risk for faster progressing ADPKD.

Each of the patients below has a combined kidney volume, also called total kidney volume (TKV), of 800 mL. Each of their kidneys is about 400 mL, which is slightly more than 13 fluid oz.

May not be at risk for fast-progressing ADPKD Height: 5’9” PATIENT 3 65 years old May not be at risk for fast-progressing ADPKD Height: 6’5” PATIENT 1 35 years old May be at risk for fast-progressing ADPKD Height: 5’9” PATIENT 2 35 years old
Height: 5’9” PATIENT 3 65 years old Height: 5’9” Height: 6’5” PATIENT 2 35 years old May be at risk for fast-progressing ADPKD PATIENT 1 35 years old May not be at risk for fast-progressing ADPKD May not be at risk for fast-progressing ADPKD

Signs of progression

You may be at greater risk for fast-progressing ADPKD if you:

  • Have high blood pressure before age 35
  • Have blood in the urine before age 35
  • Have a family history of kidney failure before age 58
  • Experienced a fast drop
    in GFR
  • Are male
  • Have certain types of inherited genetic mutations
Have you experienced any of these risk factors? See if JYNARQUE is right for you 

Progression and imaging

Work with a doctor to learn more about how your kidneys look and how fast your disease is progressing

This could include your doctor using magnetic resonance imaging (MRI) or computed tomography (CT) to get a more detailed picture of your kidneys.

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