Connective tissue diseases in primary biliary cirrhosis: A population-based cohort study

doi:10.3748/wjg.v19.i31.5131

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World Journal of Gastroenterology

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1007-9327

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World J Gastroenterol. Aug 21, 2013; 19(31): 5131-5137
Published online Aug 21, 2013. doi: 10.3748/wjg.v19.i31.5131

Table 1 Baseline characteristics of the study population

Characteristic	Cohort representation
Sex, female/male	294/28
Age (yr)	53 ± 12
Duration of disease (yr)	5.8 ± 3.5
Mayo risk score	4.5 ± 1.1
Positive ANA	275/316 (87.0)
Positive AMA	290/319 (90.9)
Positive AMA-M₂	262/290 (90.3)
Titers of AMA-M₂ (IU/mL)	139.6 ± 107.9
Liver biopsy	72 (22.4)

Data are presented as mean ± SD, ratio or n (%). ANA: Anti-nuclear antibody; AMA: Anti-mitochondrial antibody; AMA-M₂: M₂ subtype of anti-mitochondrial antibody.

Table 2 Inter-study comparison of patterns of connective tissue diseases in primary biliary cirrhosis patients n (%)

	Wang et alChina	*Watt et al[16]United Kingdom*	*Marasini et al[17]Italy*
PBC	322	160	170
CTDs in PBC	150 (46.6)	84 (53.0)	62 (36.5)
PBC + SS	121 (37.6)	40 (25.0)^a	6 (3.5)^a
PBC + SSc	9 (2.8)	12 (8.0)^a	21 (12.3)^a
PBC + SLE	12 (3.7)	2 (1.3)	3 (1.8)
PBC + RA	9 (2.8)	27 (17.0)^a	3 (1.8)
PBC + PM	10 (3.1)	0 (0.0)	1 (0.6)
PBC + MCTD	0 (0.0)	0 (0.0)	1 (0.6)

^aP < 0.05 vs Wang et al (China, the current study). PBC: Primary biliary cirrhosis; CTDs: Connective tissue diseases; SS: Sjögren’s syndrome; SSc: Systemic sclerosis; SLE: Systemic lupus erythematosus; RA: Rheumatoid arthritis; PM: Polymyositis; MCTD: Mixed connective tissue disease.

Table 3 Clinical features and laboratory results of patients with primary biliary cirrhosis alone and patients with primary biliary cirrhosis plus one other connective tissue diseases

	PBC (n =172)	PBC + SS (n =121)	PBC + SSc (n = 9)	PBC + SLE (n =12)	PBC + RA (n = 9)	PBC + PM (n = 10)
Female/male	153/19	112/9	9/0	12/0	7/2	7/3
Age (yr)	53 ± 11	53 ± 12	51 ± 6¹	50 ± 9¹	59 ± 121	53 ± 8¹
Fever	11 (6.4)	19 (15.7)^a	0 (0)	3 (25.0)	1 (11.1)	1 (10.0)
RP	32 (18.6)	23 (19.0)	4 (44.4)	5 (41.7)	0 (0)	0 (0)
Arthralgia	37 (21.5)	31 (25.6)	2 (22.2)	4 (33.3)	9 (100)^a	1 (10)
ILD	13 (7.6)	27 (22.3)^a	3 (33.3)^a	0 (0)	1 (11.1)	2 (20)
PAH	11 (6.4)	13 (10.7)	2 (22.2)	1 (8.3)	0 (0)	0 (0)
Cardiac	5 (2.9)	3 (2.5)	0 (0)	0 (0)	0 (0)	4 (40.0)^a
Renal	9 (5.2)	8 (6.6)	1 (11.1)	4 (33.3)^a	0 (0)	0 (0)
WBC (10⁹/L)	5.7 ± 2.6	5.0 ± 2.9	5.7 ± 2.8¹	4.0 ± 1.1¹^a	5.7 ± 2.4¹	7.9 ± 3.4¹^a
Hb (g/L)	126 ± 20	118 ± 18	120 ± 23¹	102 ± 20¹^a	110 ± 11¹^a	127 ± 16¹
PLT (10⁹/L)	189 ± 82	135 ± 69	190 ± 103	96 ± 75^a	247 ± 142	206 ± 9¹
ALT (U/L)	79 ± 76	91± 75	76 ± 62¹	69 ± 72¹	56 ± 46¹	73 ± 56¹
AST (U/L)	76± 62	90 ± 64	96 ± 41¹	69 ± 55¹	79 ± 52¹	73 ± 33¹
ALP (U/L)	250 ± 221	287 ± 224	291 ± 166¹	173 ± 98¹	487 ± 411¹^a	153 ± 98¹
γ-GT (U/L)	320 ± 340	309 ± 290	344 ± 346¹	207 ± 153¹^a	239 ± 166¹	264 ± 275¹
IgG (g/L)	17.1 ± 6.2	21.0 ± 12.4^a	17.3 ± 5.8¹	15.8 ± 5.2¹	22.2 ± 5.1¹^a	16.4 ± 4.4¹
IgM (g/L)	4.5 ± 4.7	4.3 ± 3.9	3.4 ± 1.8¹	1.9 ± 1.2¹^a	4.2 ± 3.6¹	5.4 ± 3.1¹
ESR (mm/1h)	41 ± 28	57 ± 38^a	47 ± 34¹	47 ± 25¹	76 ± 30¹^a	48 ± 20¹
RF+	31/156 (19.9)	92/120 (76.7)^a	4/8 (50.0)	5/11 (45.5)	9/9 (100)^a	4/10 (40.0)
ANA	142/169 (84.0)	111/119 (93.3)	9/9 (100)	12/12 (100)	8/9 (88.9)	10/10 (100)
AMA	153/171 (89.5)	109/120 (90.8)	8/9 (88.9)	11/12 (91.7)	8/9 (88.9)	10/10 (100)
AMA- M₂ (IU/mL)	147± 125	130 ± 105	119 ± 115¹	160 ± 116¹	139 ± 118¹	172 ± 138¹

Data are presented as mean ± SD, ratio or n (%).

¹Non-normally distributed data compared with PBC patients by the Wilcoxon test.

^aP < 0.05 vs PBC patients. PBC: Primary biliary cirrhosis; SS: Sjögren’s syndrome; SSc: Systemic sclerosis; SLE: Systemic lupus erythematosus; RA: Rheumatoid arthritis; PM: Polymyositis; WBC: White blood cell count; Hb: Hemoglobin; PLT: Platelet count; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; ALP: Alkaline phosphatase; γ-GT: γ-glutamyl transpeptidase; IgG: Immunoglobulin G; IgM: Immunoglobulin M; ESR: Erythrocyte sedimentation rate; RF: Rheumatoid factor; ANA: Anti-nuclear antibody; AMA: Anti-mitochondrial antibody; AMA-M₂: M₂ subtype of anti-mitochondrial antibody.

Citation: Wang L, Zhang FC, Chen H, Zhang X, Xu D, Li YZ, Wang Q, Gao LX, Yang YJ, Kong F, Wang K. Connective tissue diseases in primary biliary cirrhosis: A population-based cohort study. World J Gastroenterol 2013; 19(31): 5131-5137
URL: https://www.wjgnet.com/1007-9327/full/v19/i31/5131.htm
DOI: https://dx.doi.org/10.3748/wjg.v19.i31.5131