Cystic fibrosis patients on cystic fibrosis transmembrane conductance regulator modulators have a reduced incidence of cirrhosis

Table 1 Cystic fibrosis patient characteristics, time of enrollment and medication

Variable	CFTR modulator + ursodiol (n = 105)	CFTR modulator (n = 955)	Ursodiol (n = 529)	Neither therapy (n = 5612)
Age, mean (SD)	20.1 (12.0)	21.0 (13.0)	20.9 (13.2)	23.8 (17.3)
Age group
0-5	7	97	63	952
44723	16	142	69	671
44912	30	206	97	709
18-25	27	219	160	1079
26-34	12	148	58	709
35+	13	143	82	1492
Sex
Male	51	507	290	2613
Female	54	448	239	2999
Region
Northeast	18	190	85	1138
North central	19	260	115	1341
South	42	374	204	1997
West	24	125	109	1039
Unknown	2	6	16	97
Charlson comorbidity index, mean (SD)	0 (0.0)	0.0 (0.1)	0.0 (0.2)	0.0 (0.3)
Chronic respiratory failure	0	1	0	53
Follow up available (years), median (IQR)	2.0 (1.6-3.1)	2.1 (1.5-3.3)	2.9 (2.0-4.6)	3.0 (2.0-5.0)
Percentage of time on CFTR, median (IQR)	95% (70-100%)	100% (75-100%)	0 (0-0)	0 (0-0)
Percentage of time on ursodiol, median (IQR)	70% (23-92%)	0 (0-0)	51% (19-81%)	0 (0-0)
Percentage of time on ursodiol and CFTR Modulator concurrently, median (IQR)	56% (13-85%)	0 (0-0)	0 (0-0)	0 (0-0)

CFTR modulators: Cystic fibrosis transmembrane regulator modulators; IQR: Interquartile range; SD: Standard deviation.

Table 2 Kaplan-Meier estimates for incidence of cirrhosis at select timepoints

Time post-index	CFTR modulator + ursodiol	CFTR Modulator only	Ursodiol only	Neither therapy
3 mo	0.01	0	0.019	0.0002
6 mo	0.01	0	0.032	0.001
1 yr	0.01	0	0.059	0.001
2 yr	0.01	0	0.075	0.004
3 yr	0.01	0	0.091	0.005
4 yr	0.01	0	0.101	0.007

CFTR modulators: Cystic fibrosis transmembrane regulator modulators.