Volume Progression and Imaging Classification of Polycystic Liver in Early Autosomal Dominant Polycystic Kidney Disease

Abstract

Background and objectivesThe progression of polycystic liver disease is not well understood. The purpose of the study is to evaluate the associations of polycystic liver progression with other disease progression variables and classify liver progression on the basis of patient's age, height-adjusted liver cystic volume, and height-adjusted liver volume.Design, setting, participants, & measurementsProspective longitudinal magnetic resonance images from 670 patients with early autosomal dominant polycystic kidney disease for up to 14 years of follow-up were evaluated to measure height-adjusted liver cystic volume and height-adjusted liver volume. Among them, 245 patients with liver cyst volume >50 ml at baseline were included in the longitudinal analysis. Linear mixed models on log-transformed height-adjusted liver cystic volume and height-adjusted liver volume were fitted to approximate mean annual rate of change for each outcome. The association of sex, body mass index, genotype, baseline height-adjusted total kidney volume, and Mayo imaging class was assessed. We calculated height-adjusted liver cystic volume ranges for each specific age and divided them into five classes on the basis of annual percentage increase in height-adjusted liver cystic volume.ResultsThe mean annual growth rate of height-adjusted liver cystic volume was 12% (95% confidence interval, 11.1% to 13.1%; P<0.001), whereas that for height-adjusted liver volume was 2% (95% confidence interval, 1.9% to 2.6%; P<0.001). Women had higher baseline height-adjusted liver cystic volume than men, but men had higher height-adjusted liver cystic volume growth rate than women by 2% (95% confidence interval, 0.4% to 4.5%; P=0.02). Whereas the height-adjusted liver cystic volume growth rate decreased in women after menopause, no decrease was observed in men at any age. Body mass index, genotype, and baseline height-adjusted total kidney volume were not associated with the growth rate of height-adjusted liver cystic volume or height-adjusted liver volume. According to the height-adjusted liver cystic volume growth rate, patients were classified into five classes (number of women, men in each class): A (24, six); B (44, 13); C (43, 48); D (28, 17); and E (13, nine).ConclusionsCompared with height-adjusted liver volume, the use of height-adjusted liver cystic volume showed greater separations in volumetric progression of polycystic liver disease. Similar to the Mayo imaging classification for the kidney, the progression of polycystic liver disease may be categorized on the basis of patient’s age and height-adjusted liver cystic volume.

Beyond Loss of Kidney Function: Patient Care in Autosomal Dominant Polycystic Kidney Disease

“… 31 – 34 Growth in liver cysts declines after menopause. 35 , 36 A history of exposure to estrogen-containing oral contraceptives correlated with PLD growth in premenopausal women. Every year of exposure correlated with a 1.45% higher height-adjusted total liver volume (hTLV), which corresponded to a 15.5% higher hTLV for every 10 years of use compared with unexposed women 37 although there was no association between estrogen-containing oral contraceptive use and hTLV in the combined group of both premenopausal and postmenopausal women or in the postmenopausal subgroup.…”

“…Although there is an association with estrogen and liver cysts, two recent analyses of younger patients, either from Consortium of Radiologic Imaging Study of PKD or HALT Progression of Polycystic Kidney Disease Cohort A, did not find an independent effect of pregnancy or exogenous estrogen use on liver volume. 36 , 38 This discrepancy may be because liver cyst complications still affect only a minority of patients, and average liver volume was only slightly increased in most patients, or because other factors drive liver cyst growth. Liver cyst volume adjusted for height and age and then stratified maybe a predictive biomarker to identify individuals at highest risk for progressive liver cyst disease burden.…”

“…Liver cyst volume adjusted for height and age and then stratified maybe a predictive biomarker to identify individuals at highest risk for progressive liver cyst disease burden. 36 We discuss the use of hormonal therapy with our female patients, with a recommendation for avoidance for those at higher risk of PLD progression and individualized counseling for those at lower risk. Conservatively, imaging consistent with a total liver volume <1500 ml, or only a few cysts seen by CT or magnetic resonance imaging (MRI) imaging, maybe considered lower risk.…”

Higher need for polycystic liver disease therapy in female patients: Sex-specific association between liver volume and need for therapy

“…TLV accurately captures disease severity and progression and is unaffected by random fluctuations. [16,17] Consequently, this study aims to evaluate the association between TLV and VRT initiation in patients with PLD, with special emphasis on sex-specific differences.…”

“…We hypothesize that TLV possesses substantial prognostic value in PLD since it is the most objective parameter of disease severity. TLV accurately captures disease severity and progression and is unaffected by random fluctuations 16,17 . Consequently, this study aims to evaluate the association between TLV and VRT initiation in patients with PLD, with special emphasis on sex-specific differences.…”