Risk factors for hepatic decompensation in patients with primary biliary cirrhosis

Table 1 Demographic and clinical laboratory characteristics of primary biliary cirrhosis patients at the time of entry (n = 262) n (%)

Age (yr), mean ± SD	51.5 ± 10.2
Gender (female/male), n	240/22
The symptomatic	170 (64.9)
Jaundice	78 (29.8)
Pruritus	69 (26.3)
Persistent fatigue	63 (24.0)
Pain in the right hypochondrium	40 (15.3)
UDCA responders	190 (72.5)
Early PBC	19 (7.3)
Extra-hepatic autoimmune diseases	96 (36.6)
Sjögren's syndrome	54 (20.6)
Autoimmune thyroid diseases	25 (9.5)
CREST/systemic sclerosis	8 (3.1)/3 (1.1)
Polymyositis/dermatomyositis	8 (3.1)/1 (0.4)
Systemic lupus erythematosus	4 (1.5)
Psoriasis	4 (1.5)
Rheumatoid arthritis	3 (1.1)
Takayasu’s arteritis	2 (0.8)
Histological stages	110 (42.0)
I-II	97 (88.2)
III-IV	13 (11.8)

UDCA: Ursodeoxycholic acid; PBC: Primary biliary cirrhosis; PBC: Primary biliary cirrhosis; CREST: Comprehensive overview covers symptoms, causes, treatment of this autoimmune disorder.

Table 2 Laboratory characteristics of primary biliary cirrhosis patients at the time of entry (n = 262)

ANA	181(69.1)
AMA	245 (93.5)
ACA	73 (27.9)
ALT (IU/L)	91.0 (9-598)
AST (IU/L)	86.2 (15-428)
AST/ALT	1.10 (0.22-4.85)
ALP (IU/L)	348.5 (21-1486)
GGT (IU/L)	428.2 (9-2614)
ALB (g/L)	40.7 (25-69)
TBil (μmol/L)	28.05 (4.90-334.40)
DBil (μmol/L)	14.39 (0.34-237.40)
IgG (g/L)	16.81 (5.12-56.60)
IgA (g/L)	3.15 (0.19-8.84)
IgM (g/L)	4.66 (0.41-22.30)

Continuous data were expressed as n (%) or median (range). ANA: Anti-nuclear antibody; AMA: Anti-mitochondrial antibody; ACA: Anti-centromere antibody; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; ALP: Alkaline phosphatase; GGT: γ-glutamyl-transferase; ALB: Albumin; TBil: Total bilirubin; DBil: Direct bilirubin; Ig: Immunoglobulin.

Table 3 Complications of chronic liver disease in the primary biliary cirrhosis cohort (n = 262) n (%)

Hepatic decompensation	62 (23.7)
Portal hypertension	62 (23.7)
Hypersplenism	44 (16.8)
Esophageal varices	47 (17.9)
Variceal bleeding	35 (13.4)
Ascites	43 (16.4)
Encephalopathy	19 (7.3)
Coagulant function abnormality	12 (4.6)
Hypoproteinemia	35 (13.4)
Spontaneous bacterial peritonitis	3 (1.1)
Liver transplantation	4 (1.5)
Death-liver related	17 (6.5)1
Censor	6 (2.3)
Lost to follow-up	4 (1.5)
Death from causes unrelated to liver failure	2 (0.8)

¹One patient who underwent liver transplantation died.

Table 4 Univariate analysis of possible risk factors for hepatic decompensation in primary biliary cirrhosis

	Decompensation free (n = 196)	Decompensation (n = 62)	P value
Age at diagnosis (yr)	50.2	55.6	< 0.001
Gender (female/male)	178/18	59/3	0.275
Symptoms	60.20%	80.65%	0.003
UDCA treatment	85.20%	30.65%	< 0.001
ANA	64.80%	80.65%	0.019
AMA	92.86%	95.16%	0.731
ACA	19.39%	56.45%	< 0.001
ALT (IU/L)	89.6	96.6	0.109
AST (IU/L)	80.6	103.2	< 0.001
AST/ALT	1.05	1.27	0.013
ALP (IU/L)	311.6	454.1	< 0.001
GGT (IU/L)	395.4	524.3	0.001
ALB (g/L)	41.5	38.4	< 0.001
TBil (μmol/L)	20.92	48.84	< 0.001
DBil (μmol/L)	9.27	29.06	< 0.001
IgG (g/L)	16.61	17.43	0.386
IgA (g/L)	2.99	3.62	0.004
IgM (g/L)	4.61	4.83	0.285
Histological stage1 (I-II)	92.22%	66.67%	0.008
Early PBC	9.70%	0.00%	0.023
Extra-hepatic autoimmune disease	37.24%	37.10%	0.983

¹n = 108. ANA: Anti-nuclear antibody; AMA: Anti-mitochondrial antibody; ACA: Anti-centromere antibody; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; ALP: Alkaline phosphatase; GGT: γ-glutamyl-transferase; ALB: Albumin; TBil: Total bilirubin; DBil: Direct bilirubin; UDCA: Ursodeoxycholic acid; Ig: Immunoglobulin.

Table 5 Cox regression analysis of risk factors for hepatic decompensation in primary biliary cirrhosis (n = 262)

	HR	HR 95%CI	P value
Poor UDCA response or inconsistent treatment	4.275	2.423-7.541	< 0.001
ACA positivity	4.237	2.516-7.137	< 0.001
AAR	1.906	1.357-2.678	< 0.001
TBil	1.005	1.002-1.009	0.004
ALP	1.001	1.000-1.002	0.008

ACA: Anti-centromere antibody; ALP: Alkaline phosphatase; TBil: Total bilirubin; UDCA: Ursodeoxycholic acid; AAR: Alanine aminotransferase ratio; HR: Hazard risk.