Acquired Cystic Kidney Disease
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Renal cystic disease is a term that represents a wide spectrum of diseases that may be hereditary, developmental, or acquired; these diseases share the feature of renal cysts. These cysts can occur in the cortex, the corticomedullary junction, and/or the medulla, depending on the underlying disease process. Acquired cysts can be simple or part of acquired cystic kidney disease (ACKD), also called acquired renal cystic disease (ARCD).
Acquired cystic kidney disease is characterized by the development of numerous fluid-filled cysts in the kidneys in individuals who have no history of hereditary cystic disease. These cysts are often bilateral and are usually less than 0.5 cm in diameter but can be as large as 2-3 cm. This condition often occurs in advanced chronic kidney disease (CKD), and the cysts can antedate the clinical recognition of end-stage renal failure. In its early stages, acquired cystic kidney disease does not produce symptoms and is usually discovered incidentally in the course of abdominal imaging procedures.
For patient education information, see Renal Cell Cancer.
Acquired cystic kidney disease (ACKD) was previously thought to be a consequence of hemodialysis. Studies have shown that, although the association of dialysis and ACKD is indisputable, it is the uremic state that promotes the development of ACKD. The development of ACKD does not appear to be associated with any particular type of kidney disorder. Dialysis prolongs the patient’s survival to allow more time for acquired renal cystic disease to occur.
The exact mechanism is not known. The postulates are as follows:
Research into the cystic fluid and epithelium suggests that the cysts arise from proliferation of renal tubules. [2, 3] Cystic fluid derives from the ultrafiltrate, transepithelial solute, and fluid secretion, and resembles plasma in its composition. [2] Additionally, microdissection studies have shown that most cysts present a low cuboidal or columnar epithelium, while some of the cells that line cysts show an apical brush border configuration, suggesting that they stem from proximal tubules. [4]
Progressive destruction of the renal parenchyma triggers several mitogenic signals (eg, azotemic products, altered concentration of sodium and potassium, activation of the renin-angiotensin-system, inflammation, local growth factors), ultimately leading to hypertrophy and hyperplasia of renal tubules. [2, 5] In addition, restricted tubular fluid flow and net fluid secretion are contributory factors to form cysts. [2] Over a period of time, the activation of proto-oncogenes combined with additional risk factors may result in malignant transformation. [2, 6, 7] The image below illustrates the proposed pathophysiology of the progression of acquired cystic kidney disease to renal malignancy.
United States
The rates of occurrence of acquired cystic kidney disease are 7-22% in the predialysis population, 44% within 3 years after beginning dialysis, 79% more than 3 years after beginning dialysis, and 90% longer than 10 years after beginning dialysis. The rate of progression appears to slow after 10-15 years of dialysis.
Renal transplant recipients
The prevalence of acquired cystic kidney disease is lower in renal transplant recipients than in patients on dialysis. In a cohort of 561 renal transplant recipients, the prevalence of acquired cystic kidney disease was 23%, whereas in patients with end-stage renal disease, the prevalence may vary from 30-90%. [8] However, renal cell carcinoma develops more often in transplant recipients with acquired cystic kidney disease than in those without (19% vs. 0.5%). [8]
Acquired cystic kidney disease gives rise to many significant complications, the most serious of which is the development of renal cell neoplasms, ranging from adenoma to metastatic renal cell carcinoma. Other complications include the following:
Malignant transformation
The incidence of renal cell carcinoma (RCC) is 0.18% per year in patients with acquired cystic kidney disease, compared with 0.005% in the general population. [10] Most patients are asymptomatic, but about 15% of patients present with hematuria and flank pain; patients with metastasis may present with lumbar pain. Risk factors include the following:
Renal cancers from acquired renal cystic disease are multicentric in at least 50% of cases and bilateral in about 10% of cases. They are predominantly of the clear cell or papillary subtype. [8] The International Society of Urological Pathology Vancouver Classification of Renal Neoplasia recognizes acquired cystic disease–associated RCC as a distinct entity within the classification system. [11]
Cystic hemorrhage
Hemorrhage is sometimes associated with hematuria. Bleeding may evolve into cyst rupture, with subsequent retroperitoneal or perinephric hemorrhage (Wunderlich syndrome). Rarely, bleeding can be severe enough to cause hypovolemic shock.
Acquired cystic kidney disease is relatively more common in blacks. Blacks account for 53% of cases of acquired cystic kidney disease; however, in the United States, only 25% of patients on dialysis are black.
Acquired cystic kidney disease occurs more frequently in men than in women. Acquired cystic kidney disease–associated RCC is found predominantly in men; the male-to-female ratio is 7:1 compared with 2:1 in the general population.
Acquired cystic kidney disease occurs with equal frequency in children and adults. [12] RCC occurs approximately 20 years earlier in people with acquired cystic kidney disease than in the general population. In children, acquired cystic kidney disease–associated RCC is rare.
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Findings
ACKD
ADPKD
Kidney size
Usually not increased; may be decreased because of the advanced renal disease
Increased
Location of cysts
Cortex and medulla
Cortex and medulla
Corticomedullary differentiation*
Possible
Not possible
Normal parenchyma between cysts*
Yes
No
Extrarenal cysts (eg, liver, pancreas)
No
Yes
Positive family history
No
Yes
*On ultrasonography
Stage
Morphology
Characteristics
Percentage Malignant
Management Recommendations
Bosniak I
Simple cyst with fluid attenuation
No calcifications or septa; hairline-thin wall
~0%
No further workup needed
Bosniak II
Minimally complex cyst; diameter ≤3 cm; uniform hyperattenuation
A few hairline-thin (<1 mm) septa or thin calcifications; wall shows minimal regular thickening
~0%
No further workup needed
Bosniak IIF
Complexity intermediate between Bosniak II and III
Increased number of septa, minimally thickened with nodular or thick calcifications; contrast may produce perceptible but not measurable enhancement of septa or wall
~5%
Ultrasound/CT follow-up
Bosniak III
Complex cyst; enhanced septations or wall
Thick, nodular, irregular calcification; septa show thick, irregular, measurable enhancement with contrast
~55%
Partial nephrectomy or radiofrequency ablation in elderly or poor surgical candidates
Bosniak IV
Cystic mass; enhanced soft tissue and cyst
Thick, nodular, irregular calcification; enhanced nodule in septa and wall
~100%
Partial or total nephrectomy
Manish Suneja, MD, FASN, FACP Clinical Professor of Internal Medicine, Director, Internal Medicine Residency Program, Co-Strand Director of Clinical and Professional Skills, Dr William and Sondra Myers Professor, Department of Internal Medicine, Division of Nephrology, University of Iowa Hospitals and Clinics
Manish Suneja, MD, FASN, FACP is a member of the following medical societies: American College of Physicians, American Society of Nephrology, National Kidney Foundation
Disclosure: Editor for the book DeGowins Diagnostic examination for: McGraw Hills.
M Lee Sanders, MD, PhD, MSc Clinical Assistant Professor, University of Iowa Hospitals and Clinics; Staff Physician, Veterans Affairs Medical Center of Iowa City
M Lee Sanders, MD, PhD, MSc is a member of the following medical societies: American College of Physicians, American Society of Nephrology, American Society of Transplantation
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
George R Aronoff, MD Director, Professor, Departments of Internal Medicine and Pharmacology, Section of Nephrology, Kidney Disease Program, University of Louisville School of Medicine
George R Aronoff, MD is a member of the following medical societies: American Federation for Medical Research, American Society of Nephrology, Kentucky Medical Association, National Kidney Foundation
Disclosure: Nothing to disclose.
Vecihi Batuman, MD, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Renal Section, Southeast Louisiana Veterans Health Care System
Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology, Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.
James W Lohr, MD Professor, Department of Internal Medicine, Division of Nephrology, Fellowship Program Director, University of Buffalo State University of New York School of Medicine and Biomedical Sciences
James W Lohr, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Nephrology, Central Society for Clinical and Translational Research
Disclosure: Received research grant from: GSK<br/>Partner received salary from Alexion for employment.
Dwarakanathan Ranganathan, MD, DM, FRCP, FRACP Eminent Specialist, Head of Home, Independent Dialysis and Transition Services, Royal Brisbane and Women’s Hospital, Australia
Dwarakanathan Ranganathan, MD, DM, FRCP, FRACP is a member of the following medical societies: American Society of Nephrology, International Society for Peritoneal Dialysis, International Society of Nephrology, Australia and New Zealand Society of Nephrology, Indian Society of Nephrology
Disclosure: Nothing to disclose.
Pedro Henrique França Gois, MD Renal Fellow, Royal Brisbane and Women’s Hospital, Australia
Pedro Henrique França Gois, MD is a member of the following medical societies: American Society of Nephrology, International Society of Nephrology
Disclosure: Nothing to disclose.
Acquired Cystic Kidney Disease
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