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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2022  |  Volume : 33  |  Issue : 1  |  Page : 218-220
The Relevance of the Imaging in Autosomal Dominant Polycystic Kidney Disease Management

1 Department of Radiology and Department of Nephrology, Palermo, Italy.
2 Department of Nephrology, Dialysis, and Renal Transplantation, ARNAS Civic Hospital, Palermo, Italy.

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Date of Web Publication16-Jan-2023


How to cite this article:
Finazzo F, Cavoli GL, Mongiovì R, Bono L, Ferrantelli A, Tralongo A. The Relevance of the Imaging in Autosomal Dominant Polycystic Kidney Disease Management. Saudi J Kidney Dis Transpl 2022;33:218-20

How to cite this URL:
Finazzo F, Cavoli GL, Mongiovì R, Bono L, Ferrantelli A, Tralongo A. The Relevance of the Imaging in Autosomal Dominant Polycystic Kidney Disease Management. Saudi J Kidney Dis Transpl [serial online] 2022 [cited 2023 Jan 29];33:218-20. Available from: https://www.sjkdt.org/text.asp?2022/33/1/218/367820

To Editor,

Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic kidney disease and affects up to 12 million individuals worldwide. Due to the increased life expectancy and improved treatment of ADPKD complications, the prevalence of ADPKD has increased. Radiologic imaging is critical for successful management. Until recently, the treatments were only symptomatic but EMA in 2017 and FDA in 2018 approved Tolvaptan, a vasopressin antagonist, for ADPKD therapy. The development of cysts occurs slowly and precedes the progressive and irreversible decline in renal function; therefore, after the initial evaluation in ADPKD patients, the monitoring of the progression phase of renal damage is relevant to prevent the end-stage renal disease (ESRD).[1] Many studies show an inverse relation between glomerular filtration rate (GFR) and kidney volume; therefore, an accurate measurement of kidney size has a relevant prognostic value. Compared to other nephropathies, in ADPKD, the measurement of GFR is a less sensitive predictor biomarker of renal function, due to the slower course that allows compensatory hyperfiltration in surviving glomeruli.[2] In current nephrological practice, abdominal pain needs differential diagnosis between different complications [Figure 1]. Regarding the hemorrhage into cyst in ADPKD, the clinical presentation is not specific and computed tomography (CT) scan very often is not enough diagnostic but can rule out active bleeding. In intracystic infection in ADPKD, the symptoms are subtle and microbiological investigations do not show bacterial growth. The role of imaging in diagnosing renal neoplasia in ADPKD is challenging, but magnetic resonance imaging (MRI) with DWI and T1 fat – sat weighted images after contrast media administration can be discriminatory because the complex cysts do not often demonstrate postcontrast enhancement. Nephrologists and radiologists have to deal with many extrarenal manifestations of ADPKD that encompasses cardiovascular, gastrointestinal, and genitourinary systems. In our experience, we found often a common bile duct dilatation (>7 mm in diameter) in patients with ADPKD [Figure 2]. Ishikawa reported this finding in 22 (40%) of 55 patients suffering from ADPKD.[3] Several predicting factors associated with the progression of ADPKD toward ESRD have been investigated. In the past, standard radiographic imaging has not provided the accuracy necessary to reliably measure renal volume. With diagnostic advances, kidney volume growth is considered the principal surrogate marker predicting the decline of renal function in ADPKD; therefore, the role of total kidney volume (TKV) has been investigated as a surrogate endpoint in randomized clinical trials. TKV can be measured by ultrasound (US), CT, and MRI using manual, semi-automated, or fully automated data processing techniques. The Consortium for Radiologic Imaging Studies of Polycstic Kidney Disease (CRISP) has validated the TKV as the best prognostic biomarker of disease progression in ADPKD.[4] The CRISP experience was later used in the Mayo Clinic Translational Polycystic Kidney Disease Center to identify image-based criteria and the height-adjusted TKV (ht-TKV) determined by CT/MRI using the ellipsoid equation, producing a practical classification in typical and atypical ADPKD patients; ht-TKV and change in eGFR over time were strongly associated in typical patients.[5] These patients can be assigned into one of five subclasses (1A to 1E) with increasing rate of ht-TKV accretion in time. Sometimes, the kidney volume does not predict a change in renal function, as in patients with few large cysts or in patients with renal atrophy secondary to ischemia or urinary tract obstruction. US is the first step for ADPKD progression assessment, but its inaccuracies limit its application in clinical practice. Regarding the monitoring of ADPKD progression, a kidney with a length of >17 cm should not be considered for US examination. Precise measurements of TKV can be obtained by CT/MRI images. CT provides accurate and reliable measurements of TKV and cyst volume in ADPKD but is not routinely used for diagnosis or follow-up studies because it exposes patients to radiation. Accurate and reproducible TKV measurements, using a dose-minimizing ultra-low-dose CT protocol and volume measurement are comparable to the reference standard of MRI planimetry. Ultra-low dose CT protocol represents a viable alternative where access to MRI is limited. ERAEDTA guidelines statement recommended that MRI is used in clinical practice for identifying ADPKD patients with rapid disease progression [Figure 3]; therefore, MRI measurement of TKV is considered the gold standard for assessing the therapeutic response to new drugs developed to slow the disease progression. The imaging is essential to prevent complications from nephromegaly and massive hepatomegaly. The availability of new disease-modifying therapies will make in the near future imaging monitoring as an imperative tool in the management of ADPKD.
Figure 1: Axial T1-weighted magnetic resonance imaging section. Autosomal-dominant polycystic kidney disease: multiple cysts in both kidneys; most of them are hypointense (simple cysts) and some of them are hyperintense (blood breakdown products or proteinaceous or colloid contents). Cysts in the liver.

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Figure 2: Coronal-T2-weighted magnetic resonance imaging section. Autosomal-dominant polycystic kidney disease: enlarged kidneys (red arrows), liver cysts (blue arrows), and common bile duct (green arrow).

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Figure 3: Coronal BTFE-weighted magnetic resonance imaging section. Autosomal-dominant polycystic kidney disease: enlarged kidneys due to cysts.

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Conflict of interest: None declared.

   References Top

Chapman AB, Devuyst O, Eckardt KU, et al. Autosomal-dominant polycystic kidney disease (ADPKD): Executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int 2015;88:17-27.  Back to cited text no. 1
Gimpel C, Bergmann C, Bockenhauer D, Breysem L, Cadnapaphornchai MA, Cetiner M, et al. International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. Nat Rev Nephrol 2019;15: 713-26.  Back to cited text no. 2
Ishikawa I, Chikamoto E, Nakamura M, Asaka M, Tomosugi N, Yuri T. High incidence of common bile duct dilatation in autosomal dominant polycystic kidney disease patients. Am J Kidney Dis 1996;27:321-6.  Back to cited text no. 3
Bhutani H, Smith V, Rahbari-Oskoui F, et al. A comparison of ultrasound and magnetic resonance imaging shows that kidney length predicts chronic kidney disease in autosomal dominant polycystic kidney disease. Kidney Int 2015;88:146-51.  Back to cited text no. 4
Zhang W, Blumenfeld JD, Prince MR. MRI in autosomal dominant polycystic kidney disease. J Magn Reson Imaging 2019;50:41-51.  Back to cited text no. 5

Correspondence Address:
Dr. Gioacchino Li Cavoli
Department of Nephrology, Dialysis, and Renal Transplantation, ARNAS Civic Hospital, Palermo, Italy.

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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.367820

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