Diagnosis and Treatment Modalities of Symptomatic Polycystic Kidney Disease

ABSTRACT

Polycystic kidney disease (PKD) can cause end stage kidney disease with an autosomal dominant inheritance pattern. Besides renal replacement therapy or renal transplantation, there are no other curative therapies. Renal insufficiency, severe pain due to hemorrhagic expansion of the cysts, or infections are the most common clinical presentations. Diagnosis of infected cysts can be quite challenging. In recent years, 18FDG-PET/CT has shown to be the most sensitive and accurate modality for the diagnosis of infected cysts. The majority of these infections respond to systemic antibiotic therapy, but in some cases, percutaneous drainage is indicated. In some cases, the volume of the native polycystic kidneys is so extensive that native nephrectomy is necessary to create enough space in the iliac fossa to allow the placement of a renal graft. Tolvaptan, a selective arginine vasopressin V2 receptor antagonist, can be used to reduce the speed of disease progression in selected patients. Trans-arterial embolization has shown to be safe and effective to downsize very large native kidneys and it can be beneficial for patients who are at high risk for surgery or who decline surgery. The aim of our chapter is to present the current literature on the best diagnostic tests for patients with suspected infected or hemorrhagic cysts, and the best treatment modalities for patients with symptomatic polycystic kidneys prior or after renal transplantation.