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Imaging for the prognosis of autosomal dominant polycystic kidney disease

Nature Reviews Nephrology volume 6, pages 96–106 (2010)Cite this article

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Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the unrelenting enlargement of innumerable cysts derived from renal tubules. This cystic growth often leads to a grotesque renal enlargement. Relatively early in life, the cysts trigger secondary complications including pain, hypertension and gross hematuria; renal insufficiency is usually not detected until the fifth or sixth decade of life. Therapies targeted to molecular and pathophysiological abnormalities slow cyst growth and protect renal function in animal models of the disease. Unfortunately, the translation of these treatments into clinical trials is hampered since glomerular filtration rate, the usual biomarker of renal disease progression, does not decrease substantially until extensive and irreversible damage to noncystic parenchyma occurs. Ultrasonography, CT and MRI have been used for many years to quantify the increase in renal volume in patients with ADPKD. Imaging with these techniques has also been used to accurately quantify the rate of increased kidney and total cyst volume in patients. In this Review we discuss the overwhelming evidence in support of the view that imaging is an invaluable tool to monitor the onset and progression of ADPKD and is well-suited to gauge the response of this disease to targeted therapy before renal function begins to decline.

Key Points

  • In adult patients with autosomal dominant polycystic disease (ADPKD) and symmetrically enlarged kidneys, the magnitude of combined renal volume is a prognostic indicator of disease severity

  • Hypertension, gross hematuria and renal insufficiency seem to be linked to absolute kidney size and the rate of renal growth in patients with ADPKD

  • Imaging is an invaluable tool to monitor the progression of ADPKD and gauge response to targeted therapy in the early stages of disease

  • Ultrasonography is useful for screening individuals at risk of developing ADPKD or to follow changes over long periods of time

  • CT and MRI should be used to quantify renal volume increases over short intervals

  • Other quantitative imaging markers potentially associated with the progression of ADPKD include the size, number, spatial distribution, individual growth rate, and asymmetry of cysts

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Figure 1: MRI of a patient with autosomal dominant polycystic kidney disease.
Figure 2: Relationship between combined kidney volume and glomerular filtration rate in three cross-sectional studies.
Figure 3: Combined CT studies demonstrate that renal insufficiency (indicated by azotemia) is seen primarily in patients with the largest kidneys that grow at the fastest rates.3
Figure 4: Growth of polycystic and normal kidneys in children as determined by ultrasonography.
Figure 5: Relationship between change in glomerular filtration rate and rate of kidney growth in adults with autosomal dominant polycystic kidney disease (R = 0.20; P = 0.0022).32
Figure 6: Prognostic value of volume measurements from the CRISP study.4
Figure 7: Generalized and segmental distribution of cysts in polycystic kidney disease.
Figure 8: Asymmetric patterns of cyst distribution within and between kidneys.

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Acknowledgements

We thank C. J. Grantham for counting the cysts in MRIs of several polycystic kidneys.

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Authors and Affiliations

  1. The Department of Radiology, 200 Lothrop Street, Ste. 4895, University of Pittsburgh, Pittsburgh, 15213, PA, USA

    Kyongtae T. Bae

  2. Department of Internal Medicine, The Kidney Institute, Kansas University Medical Center, 3901 Rainbow Boulevard, Kansas City, 66160, KS, USA

    Jared J. Grantham

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Correspondence to Jared J. Grantham.

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The authors declare association with the following company: Otsuka Pharmaceutical Group as consultants.

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Bae, K., Grantham, J. Imaging for the prognosis of autosomal dominant polycystic kidney disease. Nat Rev Nephrol 6, 96–106 (2010). https://doi.org/10.1038/nrneph.2009.214

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