Prevalence and clinical characteristics of autoimmune liver disease in hospitalized patients with cirrhosis and acute decompensation in China

Table 1 Characteristics of autoimmune liver disease patients with cirrhosis and acute decompensation among different etiologies

	PBC	AIH	PBC/AIH	Others	P value
	n = 123	n = 69	n = 29	n = 21
Demographics
Age	56.00 (48.02, 63.83)	57.47 (49.33, 65.34)	52.62 (45.53, 60.85)	52.94 (41.15, 62.48)	0.28
Male, no. (%)	25 (20.3)	11 (15.9)	4 (13.8)	6 (28.6)	0.51
AD, no. (%)
Bacterial infection	34 (27.6)	21 (30.4)	13 (44.8)	8 (38.1)	0.30
Ascites	82 (66.7)	42 (60.9)	17 (58.6)	14 (66.7)	0.77
Gastrointestinal bleeding	27 (22.0)	11 (15.9)	4 (13.8)	2 (9.5)	0.42
Hepatic encephalopathy	10 (8.1)	4 (5.8)	6 (20.7)	3 (14.3)	0.11
Jaundice	61 (49.6)	35 (50.7)	19 (65.5)	9 (42.9)	0.38
Laboratory results, median (IQR)
TB, mg/dL	4.88 (1.60, 10.82)	5.15 (1.86, 11.14)	7.31 (3.04, 23.60)	4.88 (2.05, 11.72)	0.17
INR	1.29 (1.13, 1.52)	1.39 (1.23, 1.64)	1.39 (1.16, 1.49)	1.24 (1.15, 1.44)	0.044
Creatinine, mg/dL	0.65 (0.50, 0.88)	0.64 (0.51, 0.76)	0.67 (0.53, 0.79)	0.67 (0.45, 0.73)	0.51
Blood urea nitrogen, mmol/L	5.38 (3.70, 8.27)	4.73 (3.63, 5.95)	5.37 (3.90, 7.20)	4.50 (3.37, 6.60)	0.26
White blood cell, 109/L	4.39 (2.75, 6.34)	4.01 (2.81, 5.78)	6.85 (4.56, 11.76)	3.82 (3.10, 5.72)	0.001
Neutrophil-lymphocyte ratio	2.80 (1.73, 4.29)	2.00 (1.25, 3.25)	3.61 (3.01, 5.67)	2.89 (2.35, 4.86)	< 0.001
Platelet, 109/L	0.17 (0.11, 0.30)	0.15 (0.11, 0.25)	0.32 (0.14, 0.45)	0.12 (0.08, 0.30)	0.11
Albumin, g/L	30.30 (25.70, 33.70)	28.50 (25.70, 32.55)	29.90 (26.10, 33.80)	30.40 (28.45, 34.92)	0.262
ALT, IU/L	48.00 (24.50, 80.50)	59.00 (37.00, 116.00)	81.00 (61.00, 148.00)	57.90 (39.00, 85.40)	< 0.001
AST, IU/L	79.60 (48.40, 124.50)	87.00 (52.90, 187.00)	120.70 (79.00, 153.00)	90.00 (39.00, 148.00)	0.037
Hemoglobin, g/L	90.00 (68.00, 110.00)	99.00 (87.00, 119.00)	99.00 (85.00, 115.00)	98.00 (89.00, 112.00)	0.003
ALP, IU/L	208.50 (136.25, 313.00)	143.00 (105.74, 181.70)	175.50 (128.50, 243.00)	202.50 (133.50, 298.85)	< 0.001
GGT, IU/L	105.00 (51.23, 192.50)	86.63 (37.00, 126.00)	106.20 (58.70, 235.25)	122.70 (32.92, 220.22)	0.19
Pre-albumin, mg/L	78.30 (46.80, 113.90)	63.20 (40.93, 83.25)	77.30 (57.75, 97.80)	68.65 (54.75, 88.90)	0.25
Score, median (IQR)
Child Turcotte Pugh	9.00 (8.00, 10.00)	9.00 (8.00, 10.25)	10.00 (8.00, 11.00)	9.00 (8.00, 11.00)	0.58
MELD	11.00 (7.00, 17.00)	15.00 (11.00, 21.00)	18.00 (14.00, 23.00)	12.00 (10.00, 16.00)	< 0.001
MELD Na	14.00 (7.75, 19.00)	17.00 (12.00, 23.00)	20.50 (15.75, 25.00)	13.00 (10.00, 19.00)	< 0.001
CLIF SOFA	5.00 (3.00, 6.00)	5.00 (3.00, 6.00)	6.00 (3.00, 7.00)	5.00 (4.00, 6.00)	0.44

PBC: Primary biliary cirrhosis; AIH: Autoimmune hepatitis; PBC/AIH: Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome; AD: Acute decompensation; IQR: Interquartile range; TB: Total bilirubin; INR: International normalized ratio; ALT: Alanine transaminase; AST: Aspartate transaminase; ALP: Alkaline phosphatase; GGT: Gamma glutamyl transpeptidase; MELD: Model for end-stage liver disease; CLIF-SOFA: Chronic liver failure-sequential organ failure assessment.

Table 2 Characteristics of autoimmune liver disease patients with acute-on-chronic liver failure according to etiology

Variable	PBC	AIH	PBC/AIH	Others	P value
	n = 14	n = 10	n = 7	n = 3
Demographics
Age, median (IQR)	55.34 (48.88, 58.96)	55.94 (50.00, 64.01)	53.46 (47.97, 63.46)	50.85 (46.87, 58.00)	0.88
Male, no. (%)	6 (42.9)	1 (10.0)	0 (0.0)	2 (66.7)	0.04
AD, no. (%)
Bacterial infection	7 (50.0)	3 (30.0)	6 (85.7)	2 (66.7)	0.14
Ascites	9 (64.3)	6 (60.0)	5 (71.4)	3 (100.0)	0.61
Gastrointestinal bleeding	1 (7.1)	1 (10.0)	0 (0.0)	1 (33.3)	0.39
Hepatic encephalopathy	4 (28.6)	3 (30.0)	4 (57.1)	2 (66.7)	0.40
Jaundice	10 (71.4)	9 (90.0)	6 (85.7)	2 (66.7)	0.64
Laboratory results, median (IQR)
TB, mg/dL	16.78 (4.79, 27.10)	12.56 (8.94, 17.64)	23.60 (14.64, 27.52)	14.56 (8.94, 19.29)	0.71
INR	1.57 (1.34, 1.77)	2.64 (1.67, 2.80)	1.49 (1.44, 2.31)	1.50 (1.47, 1.97)	0.35
Creatinine, mg/dL	0.83 (0.79, 2.01)	0.58 (0.50, 0.74)	0.79 (0.75, 0.84)	0.68 (0.36, 1.46)	0.08
Blood urea nitrogen, mmol/L	12.05 (6.04, 20.65)	5.40 (2.74, 8.12)	7.70 (7.20, 9.25)	8.73 (6.92, 21.00)	0.09
White blood cell, 109/L	5.55 (3.62, 10.19)	4.57 (3.13, 5.34)	8.82 (6.55, 16.27)	6.71 (4.91, 9.37)	0.08
Neutrophil-lymphocyte ratio	4.55 (3.78, 5.84)	1.80 (1.06, 4.40)	7.64 (5.66, 12.77)	5.40 (4.86, 6.30)	0.05
Albumin, g/L	28.90 (26.75, 33.72)	27.80 (25.38, 31.48)	27.20 (25.05, 28.60)	27.40 (25.00, 31.15)	0.60
ALT, IU/L	105.15 (33.30, 186.90)	59.05 (38.65, 99.47)	113.90 (102.60, 204.10)	69.90 (57.55, 146.65)	0.37
AST, IU/L	121.50 (62.72, 182.23)	99.45 (61.10, 172.75)	153.00 (111.65, 238.05)	34.20 (30.30, 85.15)	0.33
Hemoglobin, g/L	88.00 (40.75, 138.00)	64.00 (33.00, 116.00)	65.00 (46.00, 77.50)	57.00 (55.20, 74.50)	0.89
ALP, IU/L	76.00 (69.00, 96.25)	86.50 (82.00, 90.75)	108.00 (89.00, 127.50)	91.40 (65.80, 102.90)	0.29
GGT, IU/L	187.00 (111.25, 252.80)	149.00 (125.65, 195.00)	153.00 (109.00, 365.00)	NA (NA, NA)	0.76
Pre-albumin, mg/L	89.00 (52.00, 119.57)	106.50 (45.25, 116.50)	63.00 (58.70, 258.60)	NA (NA, NA)	0.85
Albumin, g/L	66.50 (54.60, 115.00)	33.40 (23.55, 60.40)	87.65 (60.35, 91.55)	NA (NA, NA)	0.13
Score, median (IQR)
Child Turcotte Pugh	9.50 (8.25, 10.75)	12.00 (10.00, 12.75)	11.00 (10.50, 12.50)	11.00 (11.00, 12.50)	0.04
MELD	20.00 (18.00, 22.75)	27.50 (18.50, 28.75)	24.00 (22.00, 28.00)	21.00 (20.50, 23.00)	0.17
MELD Na	21.00 (19.00, 25.00)	26.50 (23.00, 28.75)	29.00 (23.00, 32.00)	41.00 (39.00, 41.50)	0.12
CLIF SOFA	7.00 (6.25, 8.00)	7.00 (7.00, 8.00)	8.00 (6.50, 9.00)	9.00 (8.00, 10.00)	0.32

PBC: Primary biliary cirrhosis; AIH: Autoimmune hepatitis; PBC/AIH: Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome; AD: Acute decompensation; IQR: Interquartile range; TB: Total bilirubin; INR: International normalized ratio; ALT: Alanine transaminase; AST: Aspartate transaminase; ALP: Alkaline phosphatase; GGT: Gamma glutamyl transpeptidase; MELD: Model for end-stage liver disease; CLIF-SOFA: Chronic liver failure-sequential organ failure assessment; NA: Not available.

Table 3 The impact of etiology on liver transplantation-free mortality in acute-on-chronic liver failure patients with cirrhosis and acute decompensation (primary biliary cirrhosis as a reference)

	n	Num of death (percentage)	Unadjusted OR (95%CI), P value	Adjusted I1, OR (95%CI), P value	Adjusted II2, OR (95%CI), P value
28-d LT-free mortality
PBC	111	7 (6.3)	1.0	1.0	1.0
AIH	67	4 (6.0)	0.94 (0.24-3.25), 0.93	0.94 (0.24-3.24), 0.92	1.26 (0.3-4.85), 0.74
PBC/AIH	28	5 (17.9)	3.23 (0.89-11.05), 0.06	3.43 (0.93-11.93), 0.05	2.61 (0.63-10.21), 0.17
Others	19	2 (10.5)	1.75 (0.25-7.99), 0.51	1.75 (0.24-8.13), 0.51	1.46 (0.17-8.28), 0.70
90-d LT-free mortality
PBC	103	19 (18.4)	1.0	1.0	1.0
AIH	64	8 (12.5)	0.63 (0.25-1.50), 0.31	0.64 (0.25-1.53), 0.33	0.74 (0.27-1.88), 0.54
PBC/AIH	26	7 (26.9)	1.63 (0.57-4.31), 0.34	1.70 (0.59-4.56), 0.30	1.18 (0.36-3.56), 0.77
Others	18	2 (11.1)	0.55 (0.08-2.17), 0.45	0.51 (0.08-2.02), 0.40	0.44 (0.05-2.08), 0.36
365-d LT-free mortality
PBC	97	30 (30.9)	1.0	1.0	1.0
AIH	63	16 (25.4)	0.76 (0.37-1.54), 0.45	0.76 (0.36-1.53), 0.44	0.81 (0.38-1.68), 0.58
PBC/AIH	23	11 (47.8)	2.05 (0.9-5.20), 0.13	2.06 (0.81-5.24), 0.13	1.72 (0.63-4.61), 0.28
Others	18	5 (27.8)	0.86 (0.26-2.51), 0.79	0.89 (0.26-2.62), 0.84	0.82 (0.24-2.49), 0.74

¹Adjusted age, gender.

²Adjusted age, gender, hepatic encephalopathy, ascites, infection and gastrointestinal bleeding.

OR: Odds ratio; CI: Confidence interval; PBC: Primary biliary cirrhosis; AIH: Autoimmune hepatitis; PBC/AIH: Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome; LT: Liver transplantation.

Table 4 Association of risk factors with acute-on-chronic liver failure development during hospitalization using logistic regression in univariate and multivariate analysis

	OR	95%CI	P value
Univariable logistic regression
Demographic data
Age	0.96	0.21-6.18	0.96
Male	2.89	0.83-9.17	0.08
Etiology	0.90	0.43-1.70	0.76
Acute decompensation events
Overt ascites	0.92	0.3-3.14	0.89
Gastrointestinal bleeding	0.74	0.11-2.9	0.7
Bacterial infection	2.22	0.69-6.95	0.17
Hepatic encephalopathy	3.6	0.51-15.95	0.13
Laboratory data
Total bilirubin	1.68	1.14-2.61	0.012
International normalized ratio	6.53	1.9-28.55	0.006
Creatinine	0.85	0.51-1.87	0.6
Blood urea nitrogen	1.52	0.84-2.63	0.12
Albumin	0.44	0.07-3.19	0.4
ALT	1.25	0.85-1.82	0.24
AST	1.14	0.73-1.75	0.55
White blood cells	0.88	0.45-1.75	0.72
Hemoglobin	0.39	0.11-1.5	0.16
K	1.53	0.13-18.28	0.74
Sodium	0	0-288.55	0.29
Neutrophil-lymphocyte ratio	1.37	0.84-2.23	0.21
ALP	1.15	0.59-2.27	0.7
GGT	0.86	0.54-1.34	0.52
Multivariable logistic regression
Male	3.24	0.84-11.63	0.07
TB	1.56	1.03-2.47	0.046
INR	4.31	1.15-21.70	0.048

Variables with P < 0.1 in univariate analysis were selected into multivariate analysis. Acute-on-chronic liver failure diagnosed on admission was excluded from the analysis. Continuous variables were log transformed before entering the model (base 2). ALT: Alanine transaminase; AST: Aspartate transaminase; ALP: Alkaline phosphatase; GGT: Gamma glutamyl transpeptidase; OR: Odds ratio; CI: Confidence interval; TB: Total bilirubin; INR: International normalized ratio.