Risk factors of recurrence among 471 Chinese patients with Budd-Chiari syndrome

Abstract

Budd-Chiari syndrome (BCS) is a rare form of vascular disease. There is limited literature available regarding the prognosis of this disease. The aim of this study was to characterize the cumulative recurrence rates and to investigate the risk factors of recurrence in Chinese patients with BCS. Four hundred and seventy-one patients were diagnosed as having BCS in the Affiliated Hospital of Xuzhou Medical College (Jiangsu, China) between January 2008 and December 2012. Follow-ups were conducted by phone calls or correspondence. Cumulative recurrence rates were assessed with the Kaplan-Meier curves. Independent risk factors of recurrence were calculated with the Cox's proportional hazards regression model. Four hundred and twenty-five patients with BCS had complete follow-up data, in which 24 patients died, 98 patients had recurrence, with the median duration of follow-up being 19.3 months (range 3 to 61.4). The cumulative 1-, 2-, 3-, 4- and 5-year recurrence rates were 12%, 22%, 27%, 35% and 42%, respectively. Univariable and multivariable Cox's proportional hazards regression models showed that the risk factors of recurrence include: age ≤ 30 years (HR=2.261, 95% CI: 1.412-3.621), differentiated typology (hepatic vein type: HR=1.885, 95% CI: 1.045-3.402; combined type: HR=2.088, 95% CI: 1.233-3.536), elevated lactate dehydrogenase (LDH) levels (HR=1.125, 95% CI: 1.101-1.212) and the Child-Pugh class B/C (B: HR=1.758, 95% CI: 1.057-2.926; C: HR=2.626, 95% CI: 1.396-4.940). Regardless of thrombophilia and haematological causes, exceptionally found in Chinese patients, the 5-year recurrence rate of BCS was as high as 42%. Age ≤ 30 years, hepatic vein type, combined type, increased LDH levels and the Child-Pugh class B/C were independent predictors of BCS recurrence.