Table 20.1 Type of vascular liver disease according to systemic disease a Systemic Disease Vascular liver Disease Vasculitis Behcet's disease BCS 1-3% (8-26% among angio-Behçet) Portal vein thrombosis <1% Arterial aneurysm <1% NRH < 1% PAN Cholecystis 2-17% Hepatic aneurysm <0-20%, spontaneuous rupture: Rare Connective tissue disease Anti-phospholipid syndrome BCS <1% Portal vein thrombosis <<1% PSVD <<1% HVOD <<1% Hepatic infarction << 1% (except in catastrophic APS: 3%) Lupus PSVD (NRH) 0.3-5% Hepatic arteritis (autopsy): 20% Systemic sclerosis NRH 1-4% Sarcoidosis Portal hypertension: 3% PSVD (NRH) 0-9% HVOD (sinusoidal dilatation) 0-14% BCS <<1% BCS Budd-Chiari Syndrome; PAN Periarteritis nodosa; PSVD porto-sinusoidal vascular disease; HVOD hepatic-veno-occlusive disease; NRH nodular regenerative hyperplasia a from [1-23] Table 20.2 Type of systemic disease according to vascular liver disease a