eMedicine Specialties > Urology > Congenital Urologic Conditions

Cystic Diseases of the Kidney

Author: Andrew T Trout, MD, Resident, Department of Radiology, University of Michigan
Coauthor(s): Justin Siegal, MD, Staff Physician, Department of Diagnostic Radiology, University of Washington; John M Corman, MD, Clinical Associate Professor of Urology, University of Washington at Seattle; Consulting Staff, Department of Urology, Virginia Mason Medical Center
Contributor Information and Disclosures

Updated: Jun 27, 2006

Introduction

Background

One third of people older than 50 years develop renal cysts. Although most are simple cysts, renal cystic disease has multiple etiologies. Broad categories of cystic disease include the following:

  • Developmental - Multicystic dysplastic kidney (MCDK)
  • Genetic - Autosomal recessive polycystic kidney disease (ARPKD), autosomal dominant polycystic kidney disease (ADPKD), juvenile nephronophthisis (JNPHP), medullary cystic kidney disease (MCKD), glomerulocystic kidney disease (GCKD)
  • Cysts associated with systemic disease - Von Hippel-Lindau syndrome (VHLS), tuberous sclerosis (TS)
  • Acquired - Simple cysts, acquired cystic renal disease, medullary sponge kidney (MSK)
  • Malignancy - Cystic renal cell carcinoma (RCC)
The most common larger cysts include acquired cysts, simple cysts, and cysts associated with ADPKD. Smaller cysts characterize ARPKD, JNPHP, MCKD, and MSK. In adults, renal angiomyolipomas and RCC may also have cystic components.

Pathophysiology

Cysts develop from renal tubule segments and most detach from the parent tubule after they grow to a few millimeters in size. Cyst development is generally attributed to increased proliferation of tubular epithelium, abnormalities in tubular cilia, and excessive fluid secretion.

Developmental cystic renal disease

MCDK represents abnormal development or formation of the kidney and may involve part, or all of, one or both kidneys. This condition is thought to be secondary to dysfunctional genetics, abnormal differentiation of the metanephros or in utero ureteral obstruction. Treatment is observation with surgical excision reserved for complications.

Inherited cystic renal disease

ADPKD is due to mutations in the genes PKD1 and PKD2, which encode polycystin proteins. The genetic mechanism of cyst development requires a "second hit," a somatic mutation of the normal PKD allele, which accounts for the onset of ADPKD somewhat later in life. Symptoms primarily include hypertension and renal failure, often requiring dialysis later in life. The goal of treatment is to control blood pressure and to slow the onset of renal failure. ADPKD is associated with involvement of other organs, particularly intracranial aneurysms.

ARPKD is due to mutations in PKHD1, a large gene that encodes fibrocystin/polyductin, which plays critical roles in collecting-tubule and biliary development. This disease carries a high neonatal mortality rate, and many individuals who survive eventually require renal transplantation. Symptoms include hypertension and liver disease. Diagnosis is often made in utero. Treatment is supportive in severe cases but otherwise is similar to that for ADPKD.

GCKD is often confused with ADPKD, as it is common in individuals with a family history of ADPKD. This disease is distinguished histologically and symptoms and treatment are similar to those in ADPKD.

JNPHP and medullary cystic disease are two diseases that some consider a disease complex.1 They share similar pathologic features but are due to different genetic mutations and have different inheritance patterns. JNPHP is inherited in an autosomal recessive manner and presents in childhood, while MCKD is inherited autosomal dominantly and affects adults. Both diseases present with symptoms of salt wasting and polyuria.

Systemic disease with associated renal cysts

TS is caused by mutations in the suppressor genes TSC1 and TSC2, which encode hamartin and tuberin, respectively. Renal cysts are part of a syndrome that includes seizures and dermatologic findings.

VHLS is due to mutations in the VHL gene, which increases the risk for malignancy, including RCC. Affected individuals develop cysts in multiple organs, including the kidney, pancreas, liver, and epididymis.

Acquired cystic renal disease

The exact cause of this disease is not known. It occurs exclusively in patients on dialysis. The severity of disease is directly related to the duration of therapy. Typically, acquired cystic renal disease is asymptomatic but it is known to subsequently increase the risk of RCC.

Frequency

United States

  • MCDK has an incidence of 1 per 1000-4000 live births.2
  • ADPKD has an incidence of 1 per 400-1000 persons among whites and accounts for 8-10% of all cases of end-stage renal disease (ESRD).
  • ARPKD has an incidence of 1 per 6000-55,000 live births, with a heterozygous carrier frequency of 1 per 70.
  • JNPHP affects 1 per 5000 persons.3
  • JNPHP and MCKD account for 10-20% of children with chronic renal failure and for 1-5% of all patients undergoing dialysis or transplantation.
  • TS has an incidence of 1 per 10,000-50,000 persons, and 20-25% of these patients have renal cysts.4
  • VHLS has an incidence of approximately 1 per 39,000 persons, and two thirds of these individuals develop renal cysts.
  • In acquired cystic renal disease, cysts are present in 8-13% of patients with chronic renal failure prior to dialysis. Following initiation of therapy, 10-20% of patients have acquired cystic renal disease after 3 years of dialysis, 40-60% after 5 years, and more than 90% after 10 years.5
  • MSK has an estimated incidence of 1 per 5000 persons and is found in approximately 20% of patients with nephrolithiasis.
  • Simple cysts are the most common cystic renal lesions. They are present in 5% of the general population, increasing in frequency to 25-33% of patients older than 50 years, and account for 65-70% of renal masses.
  • Cystic RCC accounts for less than 1% of RCC cases.

Mortality/Morbidity

  • Cystic renal disease accounts for approximately 10% of all ESRD cases.
  • ADPKD is 1 of the top 4 causes of ESRD and is the etiology of renal failure in 5-10% of patients undergoing dialysis. ARPKD accounts for 5% of ESRD in children.
  • Neonatal mortality secondary to ARPKD approaches 25-35% and is usually related to respiratory compromise.6
  • More than 50% of patients with ARPKD require kidney transplant before age 20 years.7
  • JNPHP is the most common cause of genetic ESRD in children.8
  • TSC is associated with a high frequency of angiomyolipoma.
  • Patients with acquired cystic disease are more likely to develop RCC (5-25%). Additionally, tumors are commonly bilateral, and 15% are metastatic.5

Race

  • ADPKD is found throughout the world in all racial and ethnic groups.
  • Acquired cystic renal disease is most common in white men and African Americans.

Sex

  • Multicystic dysplastic kidney is more common in males than in females.
  • Symptomatic progression of ADPKD appears to be more rapid in men.
  • VHLS affects men and women with equal frequency.
  • Acquired cystic renal disease is more common in men.
  • MSK has a male-to-female ratio of 2:1.

Age

  • ADPKD has a bimodal distribution of onset, with some cases presenting in infancy or childhood.3
  • ARPKD presents in infancy, childhood, or adolescence.
  • VHLS typically presents in the third or fourth decade of life with visual or central nervous system symptoms.
  • MSK typically presents between the third and fifth decades of life.
  • Simple cysts are very rare in children but increase in frequency with age.

Clinical

History

  • Developmental cystic renal disease: Multicystic dysplastic kidney (MCDK) is almost uniformly identified during prenatal sonographic examination. The involved kidney partially or completely improves with age in 40-90% of patients.9 Bilateral renal involvement is not compatible with life. MCDK can exist independently or as part of syndromes such as the vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, and radial and renal anomalies (VATER) association; Zellweger syndrome; or BOR syndrome.10
  • Inherited cystic renal disease

    • Autosomal dominant polycystic kidney disease

      • Patients present in the fourth decade of life with flank pain or intermittent hematuria. Patients may also experience cyst hemorrhage, renal infection, or nephrolithiasis. Hypertension and chronic renal failure are noted in the fifth decade of life, and patients progress to end-stage renal disease (ESRD) in the sixth decade of life.
      • The disease course varies considerably among affected individuals. While all gene carriers are believed to exhibit symptoms by the end of their eighth decade of life, only 50% of carriers actually progress to renal failure.
      • Kidney size (a direct reflection of cyst volume) increases exponentially over time and appears symmetric in a given individual, with an equal growth rate in both kidneys.11
      • All aspects of the disease appear to develop more rapidly in patients with the PKD1 genotype. The kidneys grow more rapidly in these patients, and hypertension and ESRD occur at a younger age; the onset of ESRD in persons with the PKD1 genotype occurs at a mean age of 53 years, while the onset of ESRD in persons with the PKD2 genotype occurs at a mean age of 69 years.12
      • Hepatic cysts are the most common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). These increase in number with age (20% in the third decade of life, 75% after the sixth decade of life) and may cause chronic pain. However, even with extensive cystic involvement, liver function is not compromised. Other clinical associations include cardiac valve disease (particularly mitral valve prolapse [25%]), diverticulosis, cerebral aneurysms (5-10%), pancreatic cysts, and seminal vesicle cysts.6
    • Autosomal recessive polycystic kidney disease

      • Autosomal recessive polycystic kidney disease (ARPKD) affects renal and hepatic development (dysgenesis of the portal triad), but the degree of organ involvement varies in relation to the age of onset.
      • In the neonatal period, pulmonary disease, resulting from nephromegaly and oligohydramnios, dominates the presentation. Typically, the neonate has profound respiratory compromise, often exacerbated by pneumothorax. This presentation may result in neonatal death.
      • Symptoms in an infant include hypertension (80%), diminished urine concentrating ability, and renal insufficiency. Most affected children develop hypertension within the first few years of life. Growth retardation has been reported in one fourth of children. Fifty percent of affected individuals develop ESRD in the first decade of life, requiring dialysis or transplantation.
      • In older children (4-8 y), the kidneys often are less severely affected, while hepatic disease may predominate. Hepatic involvement usually presents with symptoms secondary to portal hypertension, particularly varices and splenomegaly. Twenty-three percent of children with ARPKD experience variceal bleeding by a mean age of 12.5 years. Hepatic disease may also result in acute bacterial cholangitis or thrombocytopenia secondary to hypersplenism.
    • Glomerulocystic kidney disease (GCKD): This occurs in early (neonatal) and late (adult) forms. Neonates present with hypertension, abdominal masses, and variable degrees of renal failure. Adults typically present with flank pain, hematuria, and hypertension. Hepatic cysts may also develop.13
    • Juvenile nephronophthisis (JNPHP): This has several different phenotypic expressions depending on the gene involved. Infantile (NPHP2), juvenile (NPHP1, NPHP4) and adolescent (NPHP3) forms of the disease exist, but most symptoms appear during the first decade of life. These include growth retardation, urine concentrating defects, skeletal dysplasia, and progressive renal failure. Additionally, some degree of hepatic fibrosis and biliary duct enlargement is usually present.1
    • Medullary cystic kidney disease: This is clinically milder than JNPHP, occurs later in life (third to fourth decades), and has limited extrarenal manifestations. Individuals with this disease due to mutations in the MCKD2 gene present with uremia sooner than those with disease due to MCKD1 mutations and are more likely to develop hyperuricemia and gout.1
  • Systemic disease with associated renal cysts

    • Tuberous sclerosis (TS): Clinical features of TS include facial nevi, cardiac rhabdomyomas, epilepsy, angiofibromas, and mental retardation. Approximately one half of patients have multiple renal angiomyolipomas. Twenty to 25% of patients have renal cysts, although diffuse renal cystic disease, which may result in chronic renal failure, is rare.
    • Von Hippel-Lindau syndrome (VHLS): Clinical features of VHLS include retinal and cerebellar hemangioblastomas, pheochromocytomas, and cystic disease of the kidneys, pancreas, and epididymis. Renal cysts are very common, occurring in two thirds of patients. Renal cell carcinoma (RCC) develops in as many as 40% of patients.
  • Acquired cystic renal disease

    • Acquired renal cystic disease (ARCD): Acquired cystic disease may be found in patients with all etiologies of ESRD, particularly in patients who are dialysis-dependent. The incidence, number, and size of cysts all increase in proportion to the duration of dialysis. Most patients are asymptomatic, but symptoms may include gross hematuria, flank pain, renal colic, or a palpable renal mass. Hemorrhagic cysts occur in 50% of patients.4
    • Medullary sponge kidney (MSK) is usually detected on radiographic evaluation of adults with nephrolithiasis. Fifteen to 20% of patients with calcium oxalate and calcium phosphate renal calculi have MSK. Patients may also have a history of hematuria or urinary tract infection (UTI). Most patients with MSK, however, are asymptomatic. Approximately 10% of patients develop recurrent nephrolithiasis, bacteriuria, and pyelonephritis. Involvement is usually bilateral.
    • Simple cysts usually are clinically silent, although they occasionally hemorrhage and cause acute pain.

Physical

  • Developmental cystic renal disease: MCDK may be palpable as a flank mass in an otherwise healthy infant and is the most common cause of a renal mass and the second most common cause of a palpable abdominal mass in neonates.14,2
  • Inherited cystic renal disease

    • ARPKD: Bilateral flank masses are palpable in 30% of neonates and infants with this disease. Older children may demonstrate signs of portal hypertension.
    • ADPKD: The enlarged kidneys and liver may be palpable.
  • Acquired cystic renal disease: Simple cysts rarely become large enough to be palpable.

Causes

  • Developmental cystic renal disease: MCDK is thought to arise from abnormal development of the metanephros. This may be a genetic effect or may reflect a defect in the ampullary bud (inducer tissue) or the blastema (responder tissue), with resultant poor nephron induction.2 Additionally, in utero obstruction has been identified as a possible cause, leading to urinary stasis and cyst formation. Many patients, however, have normal renal development despite obstruction.
  • Inherited cystic renal disease: Currently, the exact mechanism of genetically induced cyst formation has not been fully defined. Similarities between cystic diseases, however, reveal common pathologic pathways. The vast majority of mutations affect the primary cilia of the tubular epithelium, indicating that disruption of this structure relates to disease development.6 Additionally, dedifferentiation and increased proliferation of tubular epithelium, along with abnormal fluid secretion, appear to be common elements in cystic disease.

    • ADPKD: Inheritance is autosomal dominant, with close to 100% penetrance. PKD1 (chromosome 16) encodes for the transmembrane protein polycystin-1 (PC1), which is responsible for cell-to-cell and cell–to–extracellular matrix binding.6 Mutations in this gene are responsible for 85-90% of cases. Mutations in polycystin-2 (PKD2, chromosome 4), a calcium channel important for PC1 localization and function, account for the remaining 10-15%.12 Interestingly, while this is a genetic disease that affects every cell in the kidney, cysts involve only 1-2% of the nephrons or collecting ducts, supporting the hypothesis that a "second hit," or mutation of the abnormal allele, must occur.1 Five to 8% of cases do not involve a family history and are the result of spontaneous mutations.
    • ARPKD: Inheritance is autosomal recessive. All cases are caused by mutations in PKHD1, a large gene that encodes fibrocystin/polyductin, which appears to be related to the polycystin complex and controls epithelial proliferation, secretion, and structure and development of the renal tubules and biliary ducts.7 The genetic defect is located on chromosome 6p21.1-p12.
    • In both ADPKD and ARPKD, epidermal growth factor (EGF) has been identified as an important stimulus for proliferation of cystic epithelium.12
    • GCKD is a rare disease that is transmitted in an autosomal dominant manner. The involved gene has not been identified, and both familial and sporadic forms exist.13
    • JNPHP is inherited in an autosomal recessive manner and is due to mutations in the NPHP genes (NPHP1-NPHP5) which are located on multiple different chromosomes and encode nephrocystins and inversin. All of the gene products are found in the primary cilium.3,12,8 Ten to 20% of cases are associated with retinal disease and are termed Senior-Loken syndrome.

      • NPHP1 is located on chromosome 2q12-13 and encodes nephrocystin.
      • NPHP2 is found on chromosome 9q22-31 and encodes inversin.
      • NPHP3 is found on chromosome 3q21-22 and encodes nephrocystin-3.
      • NPHP4 is located at chromosome 1q36 and encodes nephrocystin-4.
      • NPHP5 (chromosome 3q13.33-21.2) encodes nephrocystin-5 and is found only in cases associated with Senior-Loken syndrome.
    • Medullary cystic kidney disease (MCKD) is due to mutations in the MCKD1 (chromosome 1q21) and MCKD2 (chromosome 16p12) genes and is inherited in an autosomal dominant manner.12
  • Systemic disease with associated renal cysts

    • TS: Inheritance is autosomal dominant, with variable penetrance. Sixty to 70% of cases are due to sporadic mutations. Genetic markers have been identified at chromosome band 9q34 (TSC1, which encodes hamartin) and chromosome band 16p13 (TSC2, which encodes tuberin). TSC2 accounts for two thirds of TS cases.6,1 While the functions of these genes are not understood, TSC2 is adjacent to the PKD1 gene, which is involved in the most common form of ADPKD. In some cases, a contiguous gene syndrome has been described, involving large deletions that affect both TSC2 and PKD1.
    • VHLS: Inheritance is autosomal dominant, with variable penetrance. The genetic defect has been localized to chromosome band 3p25.
  • Recent biochemical analyses15,16 have identified a protein (mTOR) that may be the common pathway between several of the genetic forms of cystic disease. mTOR activity is related to cell growth, proliferation, apoptosis, and differentiation. Levels of mTOR have been demonstrated to be increased in cyst epithelium. Under normal conditions, PC1 (mutated in ADPKD) and TSC2 (mutated in TS) suppress or inactivate mTOR. When these genes, as well as others that relate to the primary cilia, mutate, mTOR activity becomes dysregulated, possibly allowing cyst formation. If mTOR is ultimately confirmed as a common pathway to cyst development, it will be an important drug target.
  • Acquired cystic renal disease: The exact cause of cyst formation has not been identified. One theory suggests that the development of cysts in acquired renal cystic disease (ARCD) is secondary to obstruction of the tubules by fibrosis or oxalate crystals. Another hypothesis invokes the accumulation of growth factors and stimulatory chemicals (uremia), including EGF, which leads to the development of cysts.1 The disease occurs in patients on all types of dialysis and appears to regress after transplantation.

More on Cystic Diseases of the Kidney

Overview: Cystic Diseases of the Kidney
Differential Diagnoses & Workup: Cystic Diseases of the Kidney
Treatment & Medication: Cystic Diseases of the Kidney
Follow-up: Cystic Diseases of the Kidney
Multimedia: Cystic Diseases of the Kidney
References
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Further Reading

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Keywords

acquired renal cystic disease, ARCD, acquired cystic renal disease, acquired cystic kidney disease, ACKD, dialysis-associated cystic renal disease, autosomal dominant polycystic kidney disease, ADPKD, autosomal recessive polycystic kidney disease, ARPKD, multicystic dysplastic kidney, MCDK, cystic renal dysplasia, cystic dysplasia, congenital multicystic kidney, end-stage renal disease, ESRD, medullary sponge kidney, MSK, nephronophthisis–medullary cystic kidney disease complex, NMCD, juvenile nephronophthisis, JNPHP, medullary cystic kidney disease, MCKD, nephronophthisis-uremic medullary cystic disease complex, renal cell carcinoma, RCC, tuberous sclerosis, TS, von Hippel-Lindau syndrome, VHLS, renal cysts, congenital cystic dysplasia, glomerulocystic kidney disease, GCKD

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Contributor Information and Disclosures

Author

Andrew T Trout, MD, Resident, Department of Radiology, University of Michigan
Andrew T Trout, MD is a member of the following medical societies: American Medical Association, Phi Beta Kappa, Radiological Society of North America, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Justin Siegal, MD, Staff Physician, Department of Diagnostic Radiology, University of Washington
Disclosure: Nothing to disclose.

John M Corman, MD, Clinical Associate Professor of Urology, University of Washington at Seattle; Consulting Staff, Department of Urology, Virginia Mason Medical Center
John M Corman, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Urological Association, Southwestern Oncology Group, and Western Section American Urological Association
Disclosure: Nothing to disclose.

Medical Editor

Edmund S Sabanegh, MD, Director, Center for Male Fertility, Glickman Urological and Kidney Institute, Cleveland Clinic Foundation
Edmund S Sabanegh, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Society for Reproductive Medicine, American Society of Andrology, American Urological Association, Society for the Study of Male Reproduction, Society of Reproductive Surgeons, and Southwestern Oncology Group
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

CME Editor

J Stuart Wolf, Jr, MD, FACS, David A Bloom Professor of Urology, Director, Division of Minimally Invasive Urology, Department of Urology, University of Michigan Medical Center
J Stuart Wolf, Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Medical Association, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, and Society of University Urologists
Disclosure: Terumo Corporation Consulting fee Consulting; Omeros Corporation Consulting fee Consulting

Chief Editor

Stephen W Leslie, MD, FACS, Founder and Medical Director of the Lorain Kidney Stone Research Center, Clinical Assistant Professor, Department of Urology, Medical College of Ohio
Stephen W Leslie, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, National Kidney Foundation, and Ohio State Medical Association
Disclosure: Nothing to disclose.

 
 
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