How is ADPKD treated
What are the most important treatment strategies for ADPKD?
Patients with ADPKD should be advised to adopt a healthy lifestyle. Early detection of hypertension, strict blood pressure control (130/80 mm Hg or lower) and low sodium intake (<2 g/day) are recommended because cardiovascular complications remain the most common cause of morbidity and mortality in ADPKD. Antihypertensive therapy with angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers is recommended. Dual blockade (lisinopril-telmisartan), although safe, did not show a benefit, as compared with lisinopril alone, with regard to the change in TKV or eGFR in The Polycystic Kidney Disease Treatment Network (HALT PKD) study. Protein intake should be restricted to 0.8 g/kg of ideal body weight per day. Likewise, a low cholesterol (<200 mg/day) diet is recommended in patients with hypercholesterolemia. Caffeine consumption should also be limited because it may increase cyclic adenosine monophosphate levels.
Advancements in understanding the pathophysiologic mechanisms responsible for the disease have provided a foundation for the development and testing of potential new therapies. Among them, vasopressin 2 receptor antagonist (tolvaptan) has been the most successful pharmacologic intervention at reducing the rate of TKV increase and kidney function decline in patients with ADPKD. The somatostatin analogues octreotide and lanreotide have shown a reduction in the average rate of TKV growth in the initial 6 or 12 months of treatment. Larger studies with somatostatin analogues are underway. mTOR inhibitors have been effective in slowing or arresting the progression of ADPKD in preclinical models, yet clinical trials in patients with ADPKD have been largely disappointing.
