La polykystose rénale autosomique dominante : comment et pourquoi identifier les patients "rapidement progresseurs" vers l'insuffisance rénale terminale?
[fr] La polykystose rénale autosomique dominante (PKRAD) est une maladie héréditaire fréquente qui se caractérise par le développement progressif de kystes multiples et bilatéraux dans les reins et dans d’autres organes. La majorité des patients atteints de PKRAD évolueront, à vitesses variables, vers l’insuffisance rénale terminale (IRT). Il est donc primordial de pouvoir identifier les patients «progresseurs», c’est-à-dire les patients à risque de développer précocement une IRT, afin d’optimaliser leur prise en charge. Le risque de progression peut être évalué par des critères cliniques (résumés notamment dans le PROPKD-Score), biologiques (déclin du taux de filtration glomérulaire de 4,4 à 5,9 ml/min/an et/ou doublement de la créatininémie en moins de 36 mois) ou radiologique (volume rénal total ajusté à la taille > 600 cc/m et/ou croissance annuelle > 5 %). Actuellement, il n’existe aucun traitement curatif de la PKRAD. Les antagonistes sélectifs du récepteur V2 de la vasopressine, comme le tolvaptan, ont néanmoins montré un impact bénéfique sur le ralentissement de la croissance des kystes et de la vitesse de progression vers l’IRT. La prise en charge du patient atteint de PKRAD repose essentiellement sur la correction des facteurs de progression en insistant particulièrement sur l’(hyper)hydratation, le contrôle de la pression artérielle et l’arrêt du tabagisme. [en] Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited disease characterised by the progressive development of multiple and bilateral cysts in kidneys and other organs. Most patients with ADPKD will develop, sooner or later, end-stage renal disease (ESRD). The morbidity and mortality associated with ESRD prompt physicians to identify early ADPKD patients considered as «rapid progressors», who have the greatest risk to rapidly develop ESRD. The rate of progression can be assessed by clinical - especially with the «predicting renal outcome in polycystic kidney disease score» (PROPKD-Score) -, biological (a decline of the glomerular filtration rate (GFR) of 4,4 - 5,9 ml/min/year and/or the doubling of serum creatinine within a 36-month period), or radiological criteria (total kidney volume (TKV) adjusted for the size > 600 cc/m and/or TKV annual growth rate > 5 %). Nowadays, there is no curative treatment for ADPKD. However, vasopressin-2 receptor antagonists, such as tolvaptan, appear to slow down the growth of renal cysts and the slope of GFR decline. The current management of ADPKD patients is mostly based on correcting the risk factors for progression, i.e. encouraging (over)-hydration, normalizing blood pressure, stimulating smoking cessation.
Disciplines :
Urology & nephrology Radiology, nuclear medicine & imaging
Author, co-author :
bodson, aurélie; Université de Liège - ULiège > Médecine Interne > 4e Master Médecine
MEUNIER, Paul ; Centre Hospitalier Universitaire de Liège - CHU > Service médical de radiodiagnostic
Krzesinski, Jean-Marie ; Université de Liège > Département des sciences cliniques > Néphrologie
Jouret, François ; Université de Liège > Département des sciences cliniques > Néphrologie
Language :
French
Title :
La polykystose rénale autosomique dominante : comment et pourquoi identifier les patients "rapidement progresseurs" vers l'insuffisance rénale terminale?
Alternative titles :
[en] Autosomal dominant polycystic kidney disease : how and why should we identify the patients "rapidly progressing" to end-stage renal disease?
Publication date :
01 April 2016
Journal title :
Revue Médicale de Liège
ISSN :
0370-629X
eISSN :
2566-1566
Publisher :
Université de Liège. Revue Médicale de Liège, Liège, Belgium
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