Variceal Hemorrhage and Adverse Liver Outcomes in Patients With Cystic Fibrosis Cirrhosis
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This work was financially supported by the CF Foundation grant to Dr Narkewicz, NARKEW07A0 and NIDDK grant DK062456 to Dr Molleston.
Dr Narkewicz is a consultant for AbbVie, Vertex, Gilead; stock ownership by spouse with AbbVie, Express Scripts, Gilead, Johnson & Johnson, Merck, United Health; on Speakers Bureau with CF Foundation; Grants/Contracts Research with AbbVie, CF Foundation, Vertex. Dr Leung receives grants/contracts research with AbbVie, Bristol Meyer Squibb, CF Foundation, and Gilead. Dr Karnsakul receives grants/contracts Research with CF Foundation, Gilead. Dr Murray receives grants/contracts research with Gilead; Stock ownership with Merck. Dr Schwarzenberg receives grants/contracts with AbbVie and CF Foundation. Dr Weymann: receives grants/contracts research with Novartis and funding received from Seattle Children's Hospital. Dr Molleston receives grants/contracts Research with AbbVie, Gilead, Shire. Drs Alonso, Magee, and Ye have nothing to declare.
ABSTRACT
Objectives:
Cirrhosis occurs in 5% to 10% of cystic fibrosis (CF) patients, often accompanied by portal hypertension. We analyzed 3 adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver-related death (LD), and risk factors for these in CF Foundation Patient Registry subjects with reported cirrhosis.
Methods:
We determined 10-year incidence rates for VB, LT, LD, and all-cause mortality (ACM), and examined risk factors using competing risk models and Cox-proportional hazard regression.
Results:
From 2003 to 2012, 943 participants (41% females, mean age 18.1 years) had newly reported cirrhosis; 24.7% required insulin, 85% had previous pseudomonas. Seventy-three subjects had reported VB: 38 with first VB and new cirrhosis reported simultaneously and 35 with VB after cirrhosis report. Ten-year cumulative VB, LT, and LD rates were 6.6% (95% confidence interval [CI]: 4.0, 9.1%), 9.9% (95% CI: 6.6%, 13.2%), and 6.9% (95% CI: 4.0%, 9.8%), respectively, with an ACM of 39.2% (95% CI: 30.8, 36.6%). ACM was not increased in subjects with VB compared to those without (hazard ratio [HR] 1.10, 95% CI: 0.59, 2.08). CF-related diabetes (HR: 3.141, 95% CI:1.56, 6.34) and VB (HR: 4.837, 95% CI: 2.33, 10.0) were associated with higher LT risk, whereas only worse lung function was associated with increased LD in multivariate analysis. Death rate among subjects with VB was 24% with LT and 20.4% with native liver.
Conclusions:
VB is an uncommon complication of CF cirrhosis and can herald the diagnosis, but does not affect ACM. Adverse liver outcomes and ACM are frequent by 10 years after cirrhosis report.
