Polycystic kidney disease - Treatment and prognosis

At the present stage, specific treatment of polycystic kidney disease has not been developed. Recently (early 2000), attempts have been made to develop a pathogenetic approach to treatment within the framework of an experiment, considering this pathology from the standpoint of a neoplastic process. Treatment of polycystic kidney disease in mice with antitumor drugs (paclitaxel) and a tyrosine kinase inhibitor, which inhibit cell proliferation, led to inhibition of cyst formation and a reduction in existing cysts. These treatment methods are being tested experimentally and have not yet been introduced into clinical practice.

Treatment of polycystic kidney disease in adults requires the use of a symptomatic approach and the use of drugs that inhibit the progression of chronic renal failure.

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Control of arterial hypertension

Effective control of arterial hypertension with achievement of the target blood pressure level of 130/80 mm Hg is one of the main tasks both in terms of treatment of polycystic kidney disease and in terms of prevention of rapid progression of renal failure. First-line drugs are ACE inhibitors or angiotensin 2 receptor blockers of the 1st type. They should be prescribed at the first detection of high blood pressure and taken continuously. Early use of ACE inhibitors not only allows successful control of blood pressure, but also slows down the development of renal failure. Prescribing these drugs against the background of already reduced renal function does not lead to inhibition of chronic renal failure. This is evidenced by the data of the controlled MDRD study.

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ACE inhibitors:

• captopril 25-50 mg 4 times a day;
• enalapril 2.5-20 mg/day;
• lisinopril 5-40 mg/day;
• fosinopril 10-40 mg/day;
• ramipril 1.25-20 mg/day.

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Angiotensin receptor blockers:

• losartan 25-100 mg/day;
• candesartan 4-16 mg/day;
• irbesartan 75-300 mg/day;
• telmisartan 40-80 mg/day;
• valsartan 80-320 mg/day;
• eprosartan 300-800 mg/day.

Among other antihypertensive drugs, long-acting calcium channel blockers and beta-blockers are used in polycystic kidney disease in adults. Diuretics are not indicated due to sodium pump inversion and polycythemia.

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Treatment of infected cysts

• If possible, fluid should be aspirated from an infected kidney or liver cyst.
• It is necessary to use lipophilic antibiotics with a dissociation constant that allows them to penetrate the acidic environment of the cyst within 1-2 weeks:
  • Fluoroquinolones:
    • ciprofloxacin 250-500 mg/day;
    • levofloxacin 250-500 mg/day;
    • norfloxacin 400 mg/day;
    • ofloxacin 200-400 mg/day;
  • co-trimoxazole 960 mg 2 times a day;
  • chloramphenicol 500 mg 3-4 times a day.
• If fever and purulent process progress against the background of antibiotic therapy, surgical treatment is indicated.
• In case of prolonged fever, obstruction of the urinary tract by a calculus and purulent paranephritis should be excluded.

Treatment of nephrolithiasis

• Consuming adequate amounts of fluid (at least 2 liters per day).
• Determination of metabolic changes that led to the formation of stones.
• For the prevention and treatment of the most common stones (from urates and calcium oxalates), potassium citrate is administered at a dose of 20-60 mEq/day.

Pain relief

To relieve acute pain, antispasmodics and opioids are used; in case of acute occlusion, drainage of the upper urinary tract is indicated.

For chronic pain, analgesics such as paracetamol and tramadol, tricyclic antidepressants (amitriptyline 50-150 mg/day, pipofezine 50-150 mg/day); opioids; autonomic nerve block, acupuncture are used.

If they are ineffective, invasive and surgical laparoscopic methods of treating polycystic kidney disease are used - decompression and excision of cysts, nephrectomy.

NSAIDs are not indicated for pain relief due to nephrotoxicity and the risk of decreased renal function. The introduction of sclerosing agents (alcohol) into cysts has also not been proven. Aspiration of fluid from cysts provides temporary relief, but when such procedures are repeated, the pain-free periods are sharply reduced.

Treatment of polycystic kidney disease in the stage of terminal chronic renal failure includes chronic hemodialysis and kidney transplantation. Survival of patients on hemodialysis and after kidney transplantation is practically no different from that in other chronic diffuse kidney diseases.

Prognosis of adult polycystic kidney disease

The prognosis of polycystic kidney disease is determined by the genetic variant of this disease in adults. With type 1, the prognosis is less favorable than with type 2; the prognosis of the disease is worse in males.

The prognosis for polycystic kidney disease depends on:

• the presence of arterial hypertension;
• state of kidney function;
• the rate of progression of renal failure;
• concomitant pyelonephritis;
• the presence of complications - infection of cysts, aneurysms of cerebral vessels.

In the absence of arterial hypertension and preserved renal function, the prognosis for the disease is favorable.

In the presence of renal failure, the prognosis is determined by the rate of progression of chronic renal failure, which is significantly slowed by:

• continuous monitoring of arterial hypertension, initiated at the stage of preserved renal function - target blood pressure level of 130/80 mmHg (salt restriction, use of ACE inhibitors and/or angiotensin receptor blockers);
• restriction of dietary protein to 0.8 g/kg;
• limiting fat consumption.

The prognosis of polycystic kidney disease is unfavorable in the presence of infected cysts and urinary tract infection, as well as in the presence of multiple aneurysms of the cerebral vessels.