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Nephrology > Cystic Diseases of the Kidney
Acquired Cystic Kidney Disease
Article Last Updated: Sep 15, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Dwarakanathan Ranganathan, MD, FRCP, FRACP, Senior Lecturer, Graduate Medical School, Department of Renal Medicine, University of Queensland; Senior Consultant Nephrologist, Royal Brisbane and Women's Hospital, Australia
Dwarakanathan Ranganathan is a member of the following medical societies: American Society of Nephrology, Australia and New Zealand Society of Nephrology, Indian Society of Nephrology, International Society for Peritoneal Dialysis, International Society of Nephrology, and Transplantation Society
Editors: James W Lohr, MD, Fellowship Program Director, Professor, Department of Internal Medicine, Division of Nephrology, State University of New York at Buffalo; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; George R Aronoff, MD, Director, Professor, Departments of Internal Medicine and Pharmacology, Section of Nephrology, Kidney Disease Program, University of Louisville School of Medicine; Rebecca J Schmidt, DO, FACP, FASN, Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine; Vecihi Batuman, MD, FACP, FASN, Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Medicine Service, Southeast Louisiana Veterans Health Care System
Author and Editor Disclosure
Synonyms and related keywords:
acquired cystic kidney disease, acquired renal cystic disease, renal cystic disease, renal cysts, kidney cysts, renal cell carcinoma, ACKD, ARCD
Background
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 renal cystic disease is characterized by the development of numerous fluid-filled cysts in the kidneys in individuals who have no history of hereditary cystic disease. Acquired renal cystic disease is a bilateral condition. It can antedate the clinical recognition of end-stage renal failure. In the early stages, acquired renal cystic disease does not produce symptoms and is usually discovered inadvertently in the course of abdominal imaging procedures.
Pathophysiology
Acquired renal cystic disease was thought to be a consequence of hemodialysis. Studies have shown that, although the association of dialysis and acquired renal cystic disease is indisputable, it is the uremic state that promotes the development of acquired renal cystic disease. 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: - Tubule block: The development of cysts is due to tubular abnormalities; tubular obstruction due to oxalate crystals, fibrosis, or micropolyps; and tubular fluid accumulation due to glomerular filtrate and tubular fluid excretion.
- Compensatory growth: The profound loss of renal tissue in end-stage kidney disease promotes tubular cell hypertrophy and hyperplasia. Hypertrophy and hyperplasia, together with transepithelial secretion of fluid by the tubular epithelium, result in the development of cysts. Many factors may influence the process, but most important among them are growth factors and activation of oncogenes.
- Ischemia: Kidney atrophy is a recognized consequence of ischemia that may be caused either by primary renal arterial occlusion or by the secondary arterial occlusions that develop after dialysis is begun. Parenchymal acidosis may result from chronic progressive occlusion and, if sustained just short of causing cell death, might result in renal cyst formation.
Frequency
United States
The rates of occurrence of acquired renal cystic 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.
Mortality/Morbidity
Acquired renal cystic 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. The incidence of renal cell carcinoma is 0.18% per year in patients with acquired renal cystic disease compared to 0.005% in the general population.
Race
Acquired renal cystic disease is relatively more common in blacks. Blacks account for 53% of cases of acquired renal cystic disease; however, in the United States, only 25% of patients on dialysis are black.
Sex
Acquired renal cystic disease occurs more frequently in men than in women. Acquired renal cystic disease-associated renal cell carcinoma is found predominantly in men; the male-to-female ratio is 7:1 compared with 2:1 in the general population.
Age
Acquired renal cystic disease occurs with equal frequency in children and adults. Renal cell carcinoma occurs approximately 20 years earlier in people with acquired renal cystic disease than in the general population. In children, acquired renal cystic disease-associated renal cell carcinoma is rare.
History
Acquired renal cystic disease has been described in nearly every type of renal disease that causes progressive renal insufficiency, with the exception of hereditary cystic disorders. The incidence, the number, and the size correlate to the number of years the patient is on dialysis. Acquired renal cystic disease is reversible in some patients after successful renal transplantation. Some native kidneys continue to develop cysts after transplantation, and in these patients, renal function conceivably remains significantly compromised, thereby maintaining the cystogenic state. Occasionally, the disease manifests with severe retroperitoneal or intrarenal hemorrhage, with or without hematuria, flank pain, renal colic, cyst infection, erythrocytosis, or distant metastasis from a malignant renal neoplasm. Renal cell carcinoma is the most serious complication of acquired renal cystic disease. Cysts do not develop in other organs, in contrast to autosomal dominant polycystic kidney disease (ADPKD).
Physical
Acquired renal cystic disease is usually bilateral. The kidneys are rarely palpable but may become palpable after a bleed. Comparison of Findings in ARCD Versus ADPKD
| Findings | ARCD | 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
Causes
The severity and the duration of azotemia appear to be the critical factors in determining the extent of cyst development. The causes of multiple renal cysts include the following: - Autosomal dominant polycystic kidney disease
- Autosomal recessive polycystic kidney disease
- Multicystic renal dysplasia
- Acquired renal cystic disease
- Simple renal cysts
- Medullary sponge kidney
- Familial juvenile nephronophthisis/medullary cystic disease
Polycystic Kidney Disease
Renal Cell Carcinoma
Other Problems to be Considered
When more than one renal cyst is found, consider some of the causes of cystic disorders of the kidney (see the Table in Physical).
Acquired renal cystic disease is strictly confined to the kidneys, in contrast to other hereditary cystic disorders, such as ADPKD. Consider malignant transformation.
Imaging Studies
- Ultrasonography
- Initial imaging studies include ultrasonography of the kidneys. Detecting acquired renal cystic disease in end-stage renal disease (ESRD) may be difficult using ultrasonography because of the complexity of the cysts and the increased echogenicity of the kidneys in ESRD. Differentiating between acquired renal cystic disease and ADPKD may occasionally be difficult, and the distinctive features of acquired renal cystic disease are renal size (not usually increased) and the normal parenchyma distinguishable between cysts (see the Table in Physical).
- Sonography is used more often than CT scanning as the initial screening method. Sonography is better than CT scanning to differentiate a hemorrhagic cyst because almost all hemorrhagic cysts appear isodense or slightly hyperdense on CT scanning. However, clot formation in the cystic cavity appears as an echoic mass by sonography, and dynamic CT scanning is required for differentiation of clot formation from renal cancer.
- CT scanning with contrast
- Early enhancement with contrast is superior to ultrasonography for detecting renal cancer. CT scanning is the best imaging technique to establish a diagnosis of cancer. The presence of a small renal cell carcinoma may be suspected if a CT scan shows a mass with contrast enhancement.
- Although CT scanning is useful to clinically diagnose acquired renal cystic disease, acquired cysts in patients before dialysis may be difficult to diagnose because these cysts are usually small.
- Gadolinium-enhanced magnetic resonance imaging (MRI): MRI is useful in patients who cannot tolerate CT scanning with contrast.
Other Tests
- No other test is specific to aid in the diagnosis of acquired renal cystic disease, and patients require investigations related to renal failure.
Procedures
- Fine-needle aspiration of equivocal lesions to rule out malignancy is required.
Histologic Findings
Grossly, the cysts are usually smaller than 0.5 cm in maximal dimension but may attain sizes of 2-4 cm. They affect both the renal cortex and the medulla. Many cysts contain a single layer of cuboidal epithelial cells; however, many kidneys also contain atypical cysts characterized by a multilayered epithelial lining. There appears to be a histologic continuum from cysts lined with single-layered epithelia, to those with multilayered epithelia, to renal adenoma and carcinoma. Most acquired cysts originate from the proximal tubule. Oxalate crystal deposition is predominant in the cyst walls and the renal interstitium in acquired renal cystic disease.
Staging
If renal malignancy is diagnosed, then other investigations, including chest radiography, are required to rule out distant metastasis.
Medical Care
Bleeding episodes (mild) with flank pain are treated with analgesics (eg, morphine, codeine, acetaminophen). Avoid aspirin, meperidine, and propoxyphene. Avoid heparin during hemodialysis.
Surgical Care
- Severe bleeding episodes are treated by embolization or nephrectomy.
- If carcinoma is suspected (from CT scan findings), then consider nephrectomy (cysts >3 cm in diameter and cysts <3 cm but with complications).
- Prophylactic contralateral nephrectomy is controversial; bilateral nephrectomy may be considered in those patients likely to receive kidney transplantation.
Consultations
If carcinoma is suspected in acquired renal cystic disease, consult a urologist.
Diet
No specific dietary intervention is required.
Activity
During a bleeding episode, bed rest is required.
No specific drugs are indicated in the management of acquired renal cystic disease, except analgesics for the treatment of pain. Drugs for underlying disease are required.
Drug Category: Analgesics
These agents act at the central nervous system (CNS) mu receptors and are the criterion standards for the treatment of pain resulting from kidney disease. They are inexpensive and proven effective. Disadvantages include sedation, respiratory depression, smooth muscle spasm, and the potential for abuse and addiction.
| Drug Name | Acetaminophen (Tylenol, Aspirin Free Anacin, Feverall) |
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| Description | DOC for pain in patients with documented hypersensitivity to aspirin or NSAIDs, with upper GI disease, or who are taking oral anticoagulants.
Effective in relieving mild to moderate acute pain; however, has no peripheral anti-inflammatory effects. May be preferred in elderly patients because of fewer GI and renal adverse effects. |
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| Adult Dose | 325-650 mg PO/PR q4-6h or 1000 mg tid/qid; not to exceed 4 g/d |
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| Pediatric Dose | <12 years: 10-15 mg/kg/dose PO q4-6h prn; not to exceed 2.6 g/d
>12 years: 325-650 mg PO q4h; not to exceed 4 g/d |
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| Contraindications | Documented hypersensitivity; known G-6-PD deficiency |
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| Interactions | Rifampin can reduce analgesic effects of acetaminophen; coadministration with barbiturates, carbamazepine, hydantoins, and isoniazid may increase hepatotoxicity |
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| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
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| Precautions | Hepatotoxicity possible in chronic alcoholics following various dose levels; severe or recurrent pain or high or continued fever may indicate a serious illness; contained in many OTC products and combined use with these products may result in toxicity due to cumulative doses exceeding recommended maximum dose |
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| Drug Name | Codeine |
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| Description | Indicated for moderate to severe pain. Binds to opiate receptors in CNS, causing inhibition of ascending pain pathways, altering perception and response to pain. |
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| Adult Dose | 10-60 mg/dose PO/IM/SC q4-6h prn; not to exceed 360 mg/d |
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| Pediatric Dose | 0.5 mg/kg/dose PO/IM/SC q4-6h prn; not to exceed 60 mg/dose |
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| Contraindications | Documented hypersensitivity; high altitude cerebral edema (HACE) diagnosis; elevated intracranial pressure (ICP) |
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| Interactions | Toxicity increases with concurrent administration of tricyclic antidepressants, MAO inhibitors, neuromuscular blockers, CNS depressants, phenothiazines, and narcotic analgesics |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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| Precautions | Use to treat cough in HACE diagnosed patients only if absolutely necessary; may depress hypoxic ventilatory rate and respiratory drive during sleep |
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| Drug Name | Morphine (MS Contin, MSIR, Oramorph, Duramorph) |
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| Description | DOC for analgesia because of reliable and predictable effects, safety profile, and ease of reversibility with naloxone. Various IV doses are used; commonly titrated until desired effect obtained. |
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| Adult Dose | Starting dose: 0.1 mg/kg IV/IM/SC
Maintenance dose: 5-20 mg/70 kg IV/IM/SC q4h
Relatively hypovolemic patients: Start with 2 mg IV/IM/SC; reassess hemodynamic effects of dose |
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| Pediatric Dose | Infants and children: 0.1-0.2 mg/kg dose IV/IM/SC q2-4h prn; not to exceed 15 mg/dose; may initiate at 0.05 mg/kg/dose |
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| Contraindications | Documented hypersensitivity; hypotension; potentially compromised airway where establishing rapid airway control would be difficult |
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| Interactions | Phenothiazines may antagonize analgesic effects of opiate agonists; tricyclic antidepressants, MAO inhibitors, and other CNS depressants may potentiate adverse effects of morphine |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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| Precautions | Caution in hypotension, respiratory depression, nausea, emesis, constipation, urinary retention, atrial flutter, and other supraventricular tachycardias; has vagolytic action and may increase ventricular response rate |
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Further Inpatient Care
- Patients admitted with cyst bleeding need further investigation for malignancy.
Further Outpatient Care
- If the cyst is less than 3 cm in diameter and no bleeding has occurred, follow up with imaging studies.
- If tumor enlargement is present with associated persistent hematuria, consider surgery if the patient's status permits.
Deterrence/Prevention
See related CME at Preventive Health Care in Chronic Kidney Disease and End-stage Renal Disease.
Complications
- Malignant transformation
- The risk of renal cell carcinoma is increased 40-fold in patients with acquired renal cystic disease as compared to the general population. Most patients are asymptomatic, but about 15% of patients manifest with pain, hemorrhage, and metastasis (lumbar pain). Risk factors include male sex (male-to-female ratio is 7:1), long duration of dialysis, black race, and severe acquired renal cystic disease with marked organomegaly.
- 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 papillary subtype.
- 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. Calcification can occur in or around cysts.
- Cyst infection, abscess formation, and/or sepsis
- Erythrocytosis
Prognosis
- Acquired renal cystic disease can be progressive if the patient is on dialysis for an extended period; malignant transformation may also occur. The 5-year survival rates after diagnosis of malignancy are comparable to those observed in renal cell carcinoma in the general population. Death is usually associated with metastasis and accounts for 2% of the deaths in renal transplant patients.
Patient Education
Medical/Legal Pitfalls
- Malignancy should not be overlooked in patients with acquired renal cystic disease.
Special Concerns
- The routine screening for acquired renal cystic disease in patients on dialysis is controversial. The controversy is based partly on the cost of such programs and mainly on the lack of data on the effect of screening on the patient’s outcome, including survival. The American Society of Transplantation recommends screening patients who are at high risk for renal cell carcinoma, and the European Society recommends screening patients who have been on long-term dialysis.1
- The American Society of Transplantation does not recommend screening for acquired renal cystic disease after kidney transplantation, and the European Society recommends screening when applicable. Some advocate routine screening post renal transplant for renal cell carcinoma in patients with acquired renal cystic disease.2 Screening of native kidneys in transplant recipients may be performed when they are referred for ultrasonographic evaluation of the renal allograft.
| Media file 1:
Acquired cystic kidney disease. Patient with end-stage renal disease (ESRD) on maintenance hemodialysis presented with macrohematuria. Ultrasonogram showing numerous cysts in the right kidney and previous cysts in the left kidney. |
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Media type: Image
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| Media file 2:
Acquired cystic kidney disease. A 39-year-old man on dialysis for longer than 10 years with acquired renal cystic disease (ARCD). CT scan showed a mass in the lower pole of the right kidney. Fine-needle aspiration cytology (FNAC) of the lesion showed renal cell carcinoma. The patient underwent nephrectomy. |
 | View Full Size Image | |
Media type: Image
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| Media file 3:
Acquired cystic kidney disease. A 66-year-old man with acquired renal cystic disease (ARCD) had a mass in the lower pole of the right kidney. CT-guided biopsy proved the mass to be renal cell carcinoma. This patient also had type II dissection of the aorta. |
 | View Full Size Image | |
Media type: Image
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Acquired Cystic Kidney Disease excerpt Article Last Updated: Sep 15, 2008
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