原发性胆汁性胆管炎合并干燥综合征的临床特点

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

摘要/Abstract

摘要：

目的探讨原发性胆汁性胆管炎(PBC)合并干燥综合征(SS)的临床特点。方法收集山西医科大学第二医院2010年11月-2016年6月确诊为PBC和原发性干燥综合征(pSS)的病历资料,分析患者的临床特点。结果 PBC合并SS组较单纯PBC组女性比例高、病程长;PBC合并SS患者确诊时基线资料中肝损伤相关症状及生化指标较单纯PBC患者轻;初诊诊断为PBC而后期合并SS的患者,较单纯PBC患者初诊诊断时口干症状明显;抗SSB抗体阳性率及发热症状在pSS患者较合并PBC患者多见。PBC合并SS患者和单纯PBC患者第1年和第5年肝硬化及肝功能失代偿进展情况无差异。结论 PBC合并SS患者同时具有PBC和pSS的特点,但又不完全相同,二者合并发生未加速疾病进展。

关键词: 胆管炎, 干燥综合征, 临床特点, 疾病进展

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

中图分类号:

王丹丹, 张晓岚, 邓志华. 原发性胆汁性胆管炎合并干燥综合征的临床特点[J]. 临床荟萃, 2022, 37(1): 30-34.

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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链接本文: https://huicui.hebmu.edu.cn/CN/10.3969/j.issn.1004-583X.2022.01.005

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

图/表 6

参考文献 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