Effects of Pregnancy on Liver and Kidney Cyst Growth Rates in Autosomal Dominant Polycystic Kidney Disease: A Pilot Study.

Background/Objectives: Polycystic liver disease (PLD) is the most common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). PLD is more prevalent in women, and women have larger liver cysts, possibly due to estrogen-related mechanisms. Maternal estrogen levels normally increase during pregnancy. Thus, we investigated the pregnancy-associated increase in liver volume, liver cyst volume, total kidney volume (TKV), and kidney cyst growth rates in ADPKD patients. Methods: Kidney, liver, and cyst volumes were measured in 16 ADPKD patients by magnetic resonance imaging (MRI) at multiple timepoints before and after pregnancy. The log-transformed TKV, liver volume, and cyst volume growth rates during a period with pregnancy were compared to a period without pregnancy. Results: In ADPKD patients, a higher annualized liver cyst growth rate was observed during a period with pregnancy compared to a period without pregnancy (34 ± 16%/yr vs. 23 ± 17%/yr; p-value = 0.005). Liver volume growth was also higher during a period with pregnancy, 6 [2, 7]%/yr vs. 0.3 [-0.4, 2]%/yr (p-value = 0.04). In addition, the mean kidney cyst growth rate was higher (12 ± 11%/yr vs. 4 ± 9%/yr; p-value = 0.05), and there was a trend toward a pregnancy-associated increase in the TKV growth rate (6 [4, 8]%/yr vs. 3 [0.8, 5]%/yr, (p-value = 0.14) during a period with pregnancy. Conclusions: In patients with ADPKD, the liver volume and cyst volume growth rates increased during pregnancy. This supports the hypothesis that the estrogen-mediated stimulation of liver cyst growth may contribute to the severe polycystic liver disease that is more prevalent in women than men with ADPKD. Further studies with larger populations are needed to explore the mechanisms and long-term implications of these findings.