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

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 ± 1.11a	5.7 ± 2.41	7.9 ± 3.41a
Hb (g/L)	126 ± 20	118 ± 18	120 ± 231	102 ± 201a	110 ± 111a	127 ± 161
PLT (109/L)	189 ± 82	135 ± 69	190 ± 103	96 ± 75a	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 ± 4111a	153 ± 981
γ-GT (U/L)	320 ± 340	309 ± 290	344 ± 3461	207 ± 1531a	239 ± 1661	264 ± 2751
IgG (g/L)	17.1 ± 6.2	21.0 ± 12.4a	17.3 ± 5.81	15.8 ± 5.21	22.2 ± 5.11a	16.4 ± 4.41
IgM (g/L)	4.5 ± 4.7	4.3 ± 3.9	3.4 ± 1.81	1.9 ± 1.21a	4.2 ± 3.61	5.4 ± 3.11
ESR (mm/1h)	41 ± 28	57 ± 38a	47 ± 341	47 ± 251	76 ± 301a	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.