Faculty Opinions recommendation of Blood pressure in early autosomal dominant polycystic kidney disease.

Author(s):  
Enyu Imai
Author(s):  
Young-Hwan Hwang ◽  
York Pei

Management of patients with autosomal dominant polycystic kidney disease (ADPKD) currently comprises non-specific measures including promotion of healthy lifestyle, optimization of blood pressure control, and modification of cardiovascular risk factors. A high water intake of 3–4 L per day in patients with glomerular filtration rate greater than 30 mL/min/1.73 m2 may decrease the risk of kidney stones, but its potential benefit in reducing renal cyst growth is presently unproven. Maintenance of a target blood pressure of 130/80 mmHg is recommended by expert clinical guidelines though this is unlikely to slow cyst growth. It is unclear whether pharmacological blockade of the renin–angiotensin axis confers an extrarenal protective effect. Recognition of the variable clinical presentations of cyst infection, cyst haemorrhage, or nephrolithiasis is important for early diagnosis and optimal management of these complications. Most patients with ADPKD do well on dialysis and after transplantation. Nephrectomy may be needed to make space for a donor kidney, or if kidney size or infection is an issue after end-stage renal failure is reached. Recent advances in ADPKD have led to the identification of multiple potential therapeutic targets with more than 10 clinical trials completed or currently in progress. Given the promising results of the TEMPO trial, tolvaptan may well be the first disease-modifying drug to be approved for clinical use. Several other classes of drugs (e.g. somatostatin analogues, triptolide, metformin, and glucosylceramide synthase inhibitors) with good long-term safety profiles are promising candidates which may be repurposed for this disease. In the future, identifying patients with different risks of renal disease progression by their genotype and/or kidney volume will likely assume an important role for the clinical management of ADPKD.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Magdalena Jankowska ◽  
Marek Aniserowicz ◽  
Piotr Nowak ◽  
Anna Szyndler ◽  
Michal Hoffmann ◽  
...  

Abstract Background and Aims Hypertension (AH) is an early complication of autosomal dominant polycystic kidney disease (ADPKD), which significantly increases the risk of decline of kidney function and impacts cardiovascular risk. The diagnosis of AH is often delayed and the optimal control of blood pressure (BP) is difficult to achieve in this group of patients. Of note, the optimal treatment of AH in ADPKD is yet to be established. Aim of the study was to diagnose AH (including the prevalence of masked hypertension) and to evaluate the control of BP with the use of ABPM in a cohort of ADPKD patients. Method ABPMs were performed in 163 consecutive patients, with ADPKD according to Pei criteria, appointed for the first outpatient visit. Prior to the ABPM, the diagnosis based on office BP or current AH treatment was established as well as age, sex, medication intake, and eGFR (CKD-EPI formula) were recorded. The study had a cross-sectional design. Results Out of 163 performed ABPMs, 143 were eligible for further analysis. The study group consisted of 93 females and 50 males, median age was 40 (18-87) years and median eGFR was 79.5(13-90) ml/min/1.73m2. 68% of patients had CKD G1 or 2. Median systolic blood pressure (SBP) was 127 (101-157) mmHg with blood pressure variability (BPV) 12 (7.8-23); median diastolic blood pressure (DBP) was 79 (58-98) mmHg, BPV 10.8 (6.2-17.4). 35% of patients were non-dippers, 2.7% extreme dippers and 4.9% reverse-dippers. In 31 (55%), out of 56 patients without previous diagnosis of AH, masked hypertension was found. Among 87 diagnosed with AH before the measurement, 49% were treated with 1 drug, and 29% with 2, 13% with 3, and 2% with 4. The most prevalent medication was ACE-inhibitor. Among treated, only 5.5% had all ABPM values within the target. Conclusion 55% of patients previously not diagnosed with AH on the basis of office BP proved to suffer from masked hypertension. The night DBP was the most suboptimally controlled value in ADPKD patients. Whether this is a consequence of nonadherence or suboptimal treatment, needs further investigation. ABPM is an indispensable tool in managing patients inflicted with ADPKD.


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