What is the favorable prognosis for a patient with a diagnosis of Polycystic Kidney Disease (PKD)?

From the Guidelines

For a patient with Polycystic Kidney Disease (PKD), a favorable prognosis is associated with a diagnosis of typical ADPKD, as classified by the Mayo Imaging Classification (MIC), with a height-adjusted total kidney volume indicating a slower decline in kidney function, as recommended by the kdigo 2025 clinical practice guideline 1. The prognosis of PKD is influenced by several factors, including the causal gene, type of PKD1 mutation, sex, and environmental factors.

• The causal gene (PKD1 > PKD2 or minor genes) is associated with the severity of ADPKD, with PKD1 mutations generally leading to a more severe disease course 1.
• The type of PKD1 mutation (truncating > nontruncating) also affects the severity of ADPKD, with truncating mutations leading to a more severe disease course 1.
• Sex is also a factor, with males generally having a more severe disease course than females 1.
• Environmental factors, such as obesity and salt intake, can also impact the severity of ADPKD 1. The Mayo Imaging Classification (MIC) is a useful tool for predicting future decline in kidney function and the timing of kidney failure (KF) in patients with typical ADPKD, as classified by the presence of >10 cysts on magnetic resonance imaging (MRI) or other imaging criteria 1.
• The MIC uses height-adjusted total kidney volume, adjusted for age, to stratify patients into 5 groups (1A–1E), indicating accelerating decline in kidney function 1.
• The height-adjusted total kidney volume is measured most accurately by MRI or computed tomography (CT) scan, using an automated or semiautomated tool, or alternatively, it can be estimated using the ellipsoid equation 1. A favorable prognosis is associated with a slower decline in kidney function, as indicated by a lower height-adjusted total kidney volume, and a classification of 1A or 1B on the MIC 1.
• Patients with a classification of 1A or 1B on the MIC tend to have a slower progression of disease and a lower risk of kidney failure compared to those with a classification of 1C, 1D, or 1E 1.

From the Research

Favorable Prognosis for PKD Patients

The prognosis for patients with Polycystic Kidney Disease (PKD) can be favorable if certain conditions are met. Some key factors that contribute to a favorable prognosis include:

• Early diagnosis and treatment of hypertension, which can slow the progression of renal disease in ADPKD 2
• Management of cardiovascular risk factors, such as hypertension, smoking, dyslipidemia, and obesity, which can decrease cardiovascular complications in PKD patients 2, 3
• Regular blood pressure monitoring, especially in young patients, as hypertension is a common finding in ADPKD and can occur before the impairment of renal function 4, 5

Importance of Hypertension Management

Hypertension is a significant independent risk factor for progression to end-stage renal disease (ESRD) in ADPKD patients. The pathogenesis of hypertension in ADPKD is complex and involves multiple factors, including:

• Abnormal vascular structure and function due to decreased or absent polycystin 1 or 2 expression 5
• Activation of the renin-angiotensin-aldosterone system (RAAS) due to decreased nitric oxide production and bilateral cyst expansion 5, 6
• Early vascular changes, including left ventricular hypertrophy and carotid intimal wall thickening, which can occur before the development of hypertension in ADPKD 5, 6

Multifactorial Approach to Management

A multifactorial approach to managing PKD patients is crucial, including:

• Early diagnosis and treatment of hypertension with drugs that block the RAAS 2
• Management of cardiovascular risk factors, such as smoking, dyslipidemia, and obesity 2, 3
• Regular monitoring of blood pressure and renal function to slow the progression of renal disease in ADPKD 4, 5