ADPKD: predictors of Renal Disease progression

• Published in: Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia Vol. 33; no. 5
• Main Authors: Scolari, Francesco, Dallera, Nadia, Saletti, Arianna, Terlizzi, Vincenzo, Izzi, Claudia
• Format: Journal Article
• Language: Italian
• Published: Italy 01.09.2016
• Subjects: Disease Progression; Humans; Kidney Failure, Chronic - etiology; Polycystic Kidney, Autosomal Dominant - complications; Prognosis; Risk Factors
• ISSN: 1724-5990

Factors predicting rapid progression of kidney disease in ADPKD can be divided into genetic (non-modifiable) and clinical (modifiable) risk factors. Patients harbouring PKD1 mutations, in particular if truncating, have a more severe form of ADPKD. Clinical risk factors include decrease in glomerular filtration rate and renal blood flow at a young age; high total kidney volume; hypertension and urological complications <35 years; albuminuria/proteinuria. The renal disease is also more severe in males and in subjects with family history of ESRD <55 years. In recent years, two models for predicting progression in ADPKD have been published: the Mayo model, based on height-adjusted TKV, age and eGFR, and the Brest model, based on PKD gene mutation type, gender, and early onset of hypertension and urological complications. With the emergence of new disease-modifying therapies, prediction tools are essential. However, the high variability in ADPKD makes the predicting models difficult to apply on an individual patient basis. Thus, the above-mentioned predicting models should be viewed as complimentary to clinical evaluation and follow-up. In the future, an individual risk score linking genetic, imaging and clinical data might prove the most accurate way of predicting long-term outcome.