Kidney Pain And Polycystic Kidney Disease

Pain is a common complaint in patients with autosomal-dominant polycystic kidney disease , afflicting about 60% of patients with an established diagnosis. The sensitivity of ultrasound to confirm a diagnosis of ADPKD in individuals who have a 50% a priori risk of having the disease by genetic linkage was studied by Ravine et al. Currently used criteria of bilateral cysts with at least two in one kidney provides a sensitivity of 88.5% at age 15 to 29 years and 100% at 30 years of age and above. Less stringent criteria in subjects aged 15 to 29 years to establish a diagnosis of ADPKD for individuals at risk would be at least two renal cysts (unilateral or bilateral). More stringent criteria in those aged 30 to 59 years to establish a diagnosis is the presence of at least two cysts in each kidney. For at-risk individuals 60 years and older, at least four cysts in each kidney should be required. Other reviews of ADPKD have established less exact criteria to establish a diagnosis. Although there are other etiologies of polycystic kidney disease, including autosomal-recessive disease and acquired cystic kidney disease , ADPKD is by far the most common and best studied of these cystic entities. Hence, ADPKD will be used as the prototype form of polycystic kidney disease throughout this review.

End-stage renal disease ultimately occurs in approximately 50% of patients with ADPKD by 60 years of age, and over the slow course of the disease there are a constellation of symptoms and associated findings. The spectrum of pain is the focus of this article. About 30% of ADPKD patients have accompanying polycystic liver disease upon presentation. However, the presence of liver cysts is age-dependent in ADPKD patients and may eventually be noted in up to 75 to 80% of these patients. Liver cysts are associated with various pain patterns (details below). Radiographic imaging techniques such as computed tomography (CT), ultrasound, and magnetic resonance imaging (MRI) are sensitive enough to detect cysts at a relatively early age and can help define which cysts are the largest and most amenable to approach .
Headache pain in the polycystic kidney disease population generates anxiety about the potential for a cerebral aneurysm. Detection varies between 0 and 40% of this patient group in multiple studies. However, the realistic estimate is between 4 and 6% of the total ADPKD patient group. When a family history of cerebral aneurysms is noted, the incidence of aneurysms increases 18 to 26%. Headache pain patterns will not be discussed further in this review, but it should be noted that in a survey of 184 patients with polycystic kidney disease conducted when we began to explore the issue of pain, the frequency of headaches is the same as in the general population. Chronic and migraine headaches are not related to the presence of aneurysms. In 40 polycystic kidney disease patients with severe recurring headaches, none had a detectable cerebral aneurysm by magnetic resonance angiography .
Hematuria related to cyst rupture, urinary tract infections and nephrolithiasis occur in many patients with ADPKD and the associated pain patterns are discussed in detail in this review. Severe persistent abdominal and flank pain affects patients afflicted with ADPKD and is a source of great frustration for those individuals because pain management is not well handled by many physicians. Segura et al outlined a detailed medical and surgical approach to chronic pain in renal cystic diseases, and this review is an update since their book chapter