Healthcare burden and clinical spectrum of symptomatic polycystic liver disease in Japan: a nationwide epidemiological survey.

Polycystic liver disease (PLD, defined as >10 hepatic cysts, seen with ADPKD or isolated ADPLD) is usually asymptomatic and managed with observation, ultrasound as first-line imaging (CT/MRI with volumetry for surveillance or pre-treatment planning), reassurance, avoidance of exogenous estrogen (OCP/HRT, PLD is more severe in women), and family screening; patients over 40 with only minimal hepatic involvement can generally be reassured of low progression risk. Candidacy for medical therapy is now anchored to validated symptom-burden instruments rather than a global 'symptomatic' impression: somatostatin analogs (lanreotide 120 mg or octreotide 40 mg monthly) are indicated for liver-volume-related symptoms meeting PLD-Q ≥32 or POLCA ≥14, with response (symptom relief and/or volume stabilization) reassessed at 6-12 months and therapy continued as long as benefit outweighs side effects. For SSA failure/ineligibility, management remains stepwise: cyst aspiration with sclerotherapy for a solitary dominant cyst, laparoscopic fenestration for multiple superficial cysts, partial hepatectomy for localized disease, and liver transplantation for refractory massive/end-stage disease. In ADPKD-associated PLD, combined liver-kidney transplant is recommended only when an independent indication for liver transplantation (refractory massive/end-stage PLD, as above) coexists with advanced kidney disease (eGFR <30 mL/min/1.73m²), rather than for low eGFR alone; nephrology co-management includes BP control and tolvaptan, now specifically indicated (KDIGO 2025) for eGFR ≥25 mL/min/1.73m² with high progression risk (Mayo imaging class 1C-1E, or historical eGFR decline ≥3 mL/min/1.73m²/year), titrated from 45/15 mg to a target 90/30 mg split dose.