INTRODUCTION

Autosomal dominant polycystic kidney disease (ADPKD) often leads to progressive renal failure due in part to continued enlargement of the cysts. Kidney size typically increases to more than five times normal years prior to the loss of kidney function, and measured total kidney volume is the strongest predictor for the development of renal insufficiency [1]. Other renal manifestations that can occur include hypertension, urinary tract infection, concentrating defect, hematuria, nephrolithiasis, acute or chronic flank and abdominal pain; protein excretion is generally not a prominent feature [2,3]. (See "Course and treatment of autosomal dominant polycystic kidney disease" and "Hypertension in autosomal dominant polycystic kidney disease" and "Urinary tract infection in autosomal dominant polycystic kidney disease".)

HEMATURIA

Hematuria, which is often grossly visible, occurs at some time in the course in approximately 35 to 50 percent of patients with ADPKD, usually occurs prior to loss of kidney function, and may be the presenting symptom of the disease [2,4]. A precipitating event, such as a urinary tract infection or strenuous activity, can often be identified and recurrent episodes are not uncommon [4]. Gross hematuria is associated with more rapid progression of kidney disease in ADPKD [4].

Rupture of a cyst into the collecting system is thought to be responsible for the development of hematuria. Although hemorrhage into a cyst is also common, the typical presentation is pain rather than hematuria since many cysts do not communicate with the collecting system [2]. The hematuria due to cyst rupture generally resolves within two to seven days with conservative therapy consisting of bed rest and hydration, analgesics that excluding nonsteroidals, and in some cases holding antihypertensive medications [4]. Occasionally, bleeding can persist for several weeks. With unusual and severe bleeding, percutaneous arterial embolization or even nephrectomy may become necessary [5].

Gross hematuria is more likely among individuals with larger kidneys (particularly when >15 cm in length), hypertension and higher plasma creatinine concentrations [4,6]. Frequent episodes of gross hematuria before the age of 30 may be associated with worse renal outcomes; this may possibly reflect accelerated cyst expansion [7].

Nephrolithiasis, which is described below, is another cause of hematuria, usually microscopic, in patients with ADPKD. The hematuria should resolve with passage or removal of the stone. Prolonged or recurrent hematuria, particularly in a man over the age of 50, raises the possibility of an underlying renal cell carcinoma [2] or of an unrelated problem such as bladder cancer or IgA nephropathy [8]. (See 'Renal cancer' below.)