https://doi.org/10.55788/6737c5fd
The current guidelines for clinical practice of autosomal-dominant polycystic kidney disease (ADPKD) are based on consensus statements released in 2015 [1]. So far, KDIGO guidelines have not been established on this subject. ADPKD Guideline co-chair Dr Vicente Torres (Mayo Clinic, Rochester, MN, USA) and Dr Djalila Mekahli (KU Leuven, Belgium) presented the upcoming recommendations for ADPKD diagnosis and management. Some aspects are covered in this report.
Nomenclature: For adults, following a diagnosis of ADPKD and genetic testing, KDIGO recommends a nomenclature which contains the pathogenic gene name (PKD1, PKD2, or confirmed minor gene): “ADPKD/gene”. If no genetic testing is performed or no gene is identified, the nomenclature should follow the same style [2].
Diagnosis: The initial diagnosis of ADKPD will be based on 3 diagnostic pillars as per the new KDIGO guidelines, similar to the earlier consensus statement [2]:
- ADKPD phenotype (bilateral kidney cysts, usually with kidney enlargement, often with liver cysts and other extrarenal manifestations)
- Absence of other kidney cystic disease
- With/without family history
Regarding the identification of rapidly progressing ADPKD, upcoming KDIGO guidelines recommend the Mayo Imaging Classification [3] for the prediction of decline in kidney function and evolution to kidney failure. Other markers which can be added are total kidney volume, genetic testing, ProPKD score, family history (age at kidney failure), eGFR decline, chemical biomarkers and other imaging biomarkers.
Diagnosis of paediatric ADPKD is faced with particular challenges as predictive factors for eGFR decline in children are not well established because the decline tends to be slower. The diagnostic routine is recommended similarly to adult conditions. However, kidney ultrasound is also recommended for parents in case of multiple cysts if no known family history exists. Furthermore, as hypertension is a common occurrence in paediatric ADPKD, blood pressure measurements should be carried out starting at 5 years of age. Additionally, Dr Mekahli presented a new ultrasound model called the Leuven Imaging Classification which can be used to complement the Mayo Imaging Classification but will require further validation in larger cohorts [4].
Management: The upcoming KDIGO guidelines contain general updates regarding management. Hypertension, often associated with ADPKD, and the use of lipid-lowering agents such as statins should be managed similarly to recommendations for chronic kidney disease [5]. For rapidly progressing ADPKD, tolvaptan is recommended in adults with an eGFR ≥ 25 mL/min/1.73 m2 and risk of rapid progression (based on Mayo class or eGFR decline ≥ 3 mL/min/1.73 m2/year). MTOR inhibitors, metformin, statins, sodium-glucose transport protein 2 inhibitors, somatostatin analogues and ketogenic interventions are not recommended for slowing eGFR decline [2]. At the current stage, there is insufficient clinical trial data with tolvaptan in children, so this agent is not recommended [2].
The fully updated ADPKD guidelines will be published later in 2024.
- Chapman AB, Kidney Int. 2015;88(1):17–27.
- KDIGO update - PKD guidelines 2024. Session S0.6, ERA 2024, 23–26 May, Stockholm, Sweden.
- Bais T, et al. Clin J Am Soc Nephrol. 2024;19(5):59 –601.
- Breysem L, et al. Clin J Am Soc Nephrol. 2023;18(5):581–591.
- KDIGO Working Group. Kidney Int. 2024;105(4S):S117–S314.
Medical writing support was provided by Mihai Surducan, PhD.
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