Clinical characteristic of Primary biliary cholangitis complicating Sjögren syndrome

doi:10.3969/j.issn.1004-583X.2022.01.005

Abstract

Abstract:

Objective To investigate the clinical characteristics of primary biliary cholangitis (PBC) complicating Sjögren syndrome (SS). Methods The medical records of patients diagnosed with PBC and primary Sjögren syndrome (pSS) from November 2010 to June 2016 at Second Hospital of Shanxi Medical University were collected to analyze the clinical characteristics of the patients. Results A higher proportion of females and a longer course of disease were found in PBC complicating SS group versus PBC-only group; liver injury-related symptoms and biochemical indicators in the baseline data were milder in PBC complicating SS group in comparison with PBC-only group during diagnosis confirmed; patients diagnosed with PBC at the initial diagnosis and combined with SS at a later stage had more dry mouth symptoms than those of PBC-only patients at the initial diagnosis; anti-SSB antibody positivity rate and fever symptoms were more common in patients with pSS than those with PBC combined. There was no difference in the progression of cirrhosis and hepatic decompensation between PBC complicating SS patients and PBC-only patients at year 1 and 5. Conclusion Although PBC complicating SS in patients have characteristics of both PBC and pSS, those are not identical, and the combination of the two diseases does not accelerate disease progression.

Key words: cholangitis, Sjögren syndrome, clinical characteristic, disease progression

CLC Number:

Wang Dandan, Zhang Xiaolan, Deng Zhihua. Clinical characteristic of Primary biliary cholangitis complicating Sjögren syndrome[J]. Clinical Focus, 2022, 37(1): 30-34.

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URL: https://huicui.hebmu.edu.cn/EN/10.3969/j.issn.1004-583X.2022.01.005

https://huicui.hebmu.edu.cn/EN/Y2022/V37/I1/30

Figures/Tables 6

References 14

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项目	PBC合并SS	单纯PBC	pSS	PBC^*	SS^*
例数	119	52	104	15	12
性别(男/女)	2/117	5/47^#	3/101	0/15	0/12
年龄(岁)	57.15±9.20	58.31±11.23	54.54±12.38	56.22±4.30	52.00±6.21
病程(月)	65.31±74.46	26.38±42.75^#	58.87±69.40	42.22±34.95	80.22±77.75
ESR(mm/h)	43.01±29.90	45.14±30.74	46.62±36.58	41.07±8.57	48.08±28.12
CRP(mg/dl)	7.07±11.56	17.75±27.25	13.85±27.08	10.97±4.20	17.11±45.15
静脉曲张[例(%)]	28/40(70.00)	23/34(67.65)		8/14(57.14)
上消化道出血[例(%)]	3/119(2.52)	5/52(9.62)		0/15(0.00)
腹水[例(%)]	16/95(16.84)	14/41(34.15)^#		3/14(21.43)
肝硬化[例(%)]	62/119(52.10)	40/52(76.92)^#		5/15(33.33)^△
肝功能失代偿[例(%)]	29/119(24.37)	30/52(57.69)^#		3/15(20.00)^△

项目	PBC合并SS	单纯PBC	pSS	PBC^*	SS^*
例数	119	52	104	15	12
性别(男/女)	2/117	5/47^#	3/101	0/15	0/12
年龄(岁)	57.15±9.20	58.31±11.23	54.54±12.38	56.22±4.30	52.00±6.21
病程(月)	65.31±74.46	26.38±42.75^#	58.87±69.40	42.22±34.95	80.22±77.75
ESR(mm/h)	43.01±29.90	45.14±30.74	46.62±36.58	41.07±8.57	48.08±28.12
CRP(mg/dl)	7.07±11.56	17.75±27.25	13.85±27.08	10.97±4.20	17.11±45.15
静脉曲张[例(%)]	28/40(70.00)	23/34(67.65)		8/14(57.14)
上消化道出血[例(%)]	3/119(2.52)	5/52(9.62)		0/15(0.00)
腹水[例(%)]	16/95(16.84)	14/41(34.15)^#		3/14(21.43)
肝硬化[例(%)]	62/119(52.10)	40/52(76.92)^#		5/15(33.33)^△
肝功能失代偿[例(%)]	29/119(24.37)	30/52(57.69)^#		3/15(20.00)^△

症状	PBC合并SS	单纯PBC	pSS	PBC^*	SS^*
口干	92/119(77.31)	13/52(25.00)^#	86/104(82.69)	9/15(60.00)^△	12/12(100.00)
眼干	60/119(50.42)	6/52(11.54)^#	58/104(55.77)	5/15(33.33)	9/12(75.00)
牙齿脱落	32/119(26.89)	2/52(3.85)^#	39/104(37.50)	2/15(13.33)	3/12(25.00)
腮腺肿大	4/119(3.36)	1/52(1.92)	6/104(5.77)	1/15(6.67)	1/12(8.33)
关节痛	44/119(36.97)	5/52(9.62)^#	42/104(40.38)	1/15(6.67)	6/12(50.00)
发热	6/119(5.04)	0/52(0.00)	16/104(15.38)^#	1/15(6.67)	0/12(0.00)
口腔溃疡	14/119(11.76)	0/52(0.00)^#	10/104(9.62)	1/15(6.67)	2/12(16.67)
雷诺现象	4/119(3.36)	1/52(1.92)	6/104(5.77)	0/15(0.00)	1/12(8.33)
乏力	39/119(32.77)	30/52(57.69)^#	16/104(15.38)^#	7/15(46.67)	1/12(8.33)
瘙痒	17/119(14.29)	17/52(32.69)^#	7/104(6.73)	2/15(13.33)	2/12(16.67)
黄疸	10/119(8.40)	14/52(26.92)^#	2/104(1.92)^#	3/15(20.00)	0/12(0.00)
腹部不适	22/119(18.49)	17/52(32.69)^#	2/104(1.92)^#	4/15(26.67)	1/12(8.33)
纳差	19/119(15.97)	6/52(11.54)	4/104(3.85)^#	4/15(26.67)	0/12(0.00)
呕血黑便	2/119(1.68)	6/52(11.54)^#	0/104(0.00)	0/15(0.00)	0/12(0.00)
肝大	3/93(3.23)	1/40(2.50)	1/62(1.61)	0/15(0.00)	0/12(0.00)
脾大	52/93(55.91)	26/41(63.41)	4/62(6.45)^#	4/15(26.67)	0/12(0.00)

症状	PBC合并SS	单纯PBC	pSS	PBC^*	SS^*
口干	92/119(77.31)	13/52(25.00)^#	86/104(82.69)	9/15(60.00)^△	12/12(100.00)
眼干	60/119(50.42)	6/52(11.54)^#	58/104(55.77)	5/15(33.33)	9/12(75.00)
牙齿脱落	32/119(26.89)	2/52(3.85)^#	39/104(37.50)	2/15(13.33)	3/12(25.00)
腮腺肿大	4/119(3.36)	1/52(1.92)	6/104(5.77)	1/15(6.67)	1/12(8.33)
关节痛	44/119(36.97)	5/52(9.62)^#	42/104(40.38)	1/15(6.67)	6/12(50.00)
发热	6/119(5.04)	0/52(0.00)	16/104(15.38)^#	1/15(6.67)	0/12(0.00)
口腔溃疡	14/119(11.76)	0/52(0.00)^#	10/104(9.62)	1/15(6.67)	2/12(16.67)
雷诺现象	4/119(3.36)	1/52(1.92)	6/104(5.77)	0/15(0.00)	1/12(8.33)
乏力	39/119(32.77)	30/52(57.69)^#	16/104(15.38)^#	7/15(46.67)	1/12(8.33)
瘙痒	17/119(14.29)	17/52(32.69)^#	7/104(6.73)	2/15(13.33)	2/12(16.67)
黄疸	10/119(8.40)	14/52(26.92)^#	2/104(1.92)^#	3/15(20.00)	0/12(0.00)
腹部不适	22/119(18.49)	17/52(32.69)^#	2/104(1.92)^#	4/15(26.67)	1/12(8.33)
纳差	19/119(15.97)	6/52(11.54)	4/104(3.85)^#	4/15(26.67)	0/12(0.00)
呕血黑便	2/119(1.68)	6/52(11.54)^#	0/104(0.00)	0/15(0.00)	0/12(0.00)
肝大	3/93(3.23)	1/40(2.50)	1/62(1.61)	0/15(0.00)	0/12(0.00)
脾大	52/93(55.91)	26/41(63.41)	4/62(6.45)^#	4/15(26.67)	0/12(0.00)

项目	PBC合并SS	单纯PBC	pSS	PBC^*	SS^*
ALT(U/L)	71.56±66.45	98.48±94.11^#	30.35±28.20^#	76.07±10.01	33.50±19.83
AST(U/L)	72.75±54.89	117.26±90.31^#	30.27±18.31^#	85.07±9.95	32.00±17.71
ALP(U/L)	228.77±160.33	402.36±263.73^#	76.72±49.93^#	304.53±71.11	71.50±20.68
GGT(U/L)	237.52±229.03	379.63±325.08^#	33.60±44.93^#	207.33±48.32	18.75±10.10
TBIL(μmol/L)	23.07±20.69	50.80±80.11^#	12.25±4.94^#	41.23±29.59	10.08±3.18
ALB(g/L)	36.97±7.10	32.19±6.37^#	37.22±5.01	32.67±3.15	39.92±1.56
TC(mmol/L)	5.17±1.95	4.72±1.69	4.41±1.06^#	4.11±0.49	4.42±0.35
AMA[例(%)]	51/69(73.91)	20/32(62.50)	2/33(6.06)^#	7/10(70.00)	0/10(0.00)
AMA-M2[例(%)]	106/113(93.81)	41/48(85.42)	2/38(5.26)^#	14/14(100.00)	0/10(0.00)
抗GP210[例(%)]	17/91(18.68)	11/45(24.44)	0/36(0.00)^#	3/14(21.43)	0/10(0.00)
抗SP100[例(%)]	9/91(9.89)	9/45(20.00)	1/36(2.78)	2/14(14.29)	0/10(0.00)
ANA[例(%)]	93/104(89.42)	30/32(93.75)	86/95(90.53)	7/10(70.00)	9/10(90.00)
抗SSA抗体[例(%)]	74/102(72.55)	6/32(18.75)^#	78/95(82.11)	1/10(10.00)	9/10(90.00)
抗SSB抗体[例(%)]	5/102(4.91)	0/32(0.00)	15/95(15.79)^#	0/10(0.00)	1/10(10.00)
IgG(g/L)	16.03±5.82	15.04±4.46	18.21±10.18	14.53±2.39	15.45±2.89
IgA(g/L)	3.27±1.41	3.36±1.42	2.99±1.34	3.38±0.45	2.70±0.45
IgM(g/L)	3.74±2.39	3.76±1.64	1.49±0.86^#	3.55±0.25	1.42±0.39