Autosomal dominant polycystic kidney disease (
ADPKD) is a dominantly inherited systemic disorder equally inherited in men and women characterized by renal
cyst development and expansion ultimately leading to
renal failure.
ADPKD women have a slower rate of progression to
renal failure, with a later age of entry into
end-stage renal disease (
ESRD) as compared with men. Renal
cyst growth and renal expansion are the hallmarks of
ADPKD, and women will develop
renal insufficiency with smaller renal volume than their male counterparts. As well, women have different rates of occurrence of renal and extrarenal complications in
ADPKD. Renal complications related to
ADPKD, including
hypertension and gross
hematuria, occur more frequently in men than in women, whereas liver cystic disease occurs earlier and more frequently in women than in men. The presence of
polycystic liver disease is related to pregnancy number and
oral contraceptive pill use in
ADPKD women. Importantly, massive
polycystic liver disease requiring surgical intervention occurs primarily in
ADPKD women. ADKPD women have a highly successful reproductive course. The chance of a successful pregnancy is excellent in
ADPKD women and comparable to healthy unaffected women as long as prepregnancy blood pressure and renal function are normal. Fetal complication rates are no greater than in the general population; however, maternal complication rates in
ADPKD women are high with an increased frequency of new or worsening
hypertension as well as an increased occurrence of
preeclampsia and preterm deliveries. Finally, increasing pregnancy number has minimal or no effect on renal outcome in
ADPKD women.