门脉肝窦血管病的研究进展

何福亮; 贾继东

doi:10.12449/JCH240103

门脉肝窦血管病的研究进展

DOI: 10.12449/JCH240103

何福亮^, ,,
贾继东

首都医科大学附属北京友谊医院肝病中心，北京 100050

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：何福亮负责查阅文献，撰写论文；贾继东负责拟定文章思路，修改并最终定稿。

详细信息

通信作者:
何福亮， hefuliangjiayuan@163.com （ORCID： 0000-0003-1643-1465）

计量
- 文章访问数: 985
- HTML全文浏览量: 141
- PDF下载量: 239
- 被引次数: 0
出版历程
- 收稿日期: 2023-12-14
- 录用日期: 2023-12-20
- 出版日期: 2024-01-23

Research advances in porto-sinusoidal vascular disease

Fuliang HE^{,
,},
Jidong JIA

Liver Research Center，Beijing Friendship Hospital，Capital Medical University，Beijing 100050，China

More Information

Corresponding author: HE Fuliang， hefuliangjiayuan@163.com （ORCID： 0000-0003-1643-1465）

摘要

摘要: 门脉肝窦血管病（PSVD）是近年提出的新的疾病命名，是特发性非硬化性门静脉高压的重要补充，纳入了病理符合但不合并门静脉高压症状、合并门静脉血栓及合并病毒性肝炎等患者。本文对门脉肝窦血管性疾病的命名、流行病学、病因学、临床表现、预后及治疗进行阐述，以期提高临床医生对该病的认识。
- 门静脉肝窦血管病 /
- 预后 /
- 治疗学
Abstract: Porto-sinusoidal vascular disease （PSVD） is a new disease nomenclature proposed in recent years， which is an important supplement to idiopathic non-cirrhotic portal hypertension. PSVD includes the patients with specific pathological conditions， but without portal hypertension symptoms， and the patients with portal vein thrombosis or viral hepatitis. This article elaborates on the naming， epidemiology， etiology， clinical manifestations， prognosis， and treatment of PSVD， in order to improve the understanding of this disease among clinicians.
- Porto-Sinusoidal Vascular Disease /
- Prognosis /
- Therapeutics

HTML全文

表 1 PSVD的诊断条件

Table 1. Diagnostic conditions of PSVD

肝活检病理无肝硬化，标本长度≥20 mm	+	符合1条门静脉高压特征性表现或符合1条PSVD病理表现
或
肝活检病理无肝硬化，标本长度≥20 mm	+	符合1条门静脉高压非特征性表现和符合1条PSVD病理表现

下载: 导出CSV

表 2 PSVD的临床及病理特征

Table 2. The clinical and pathological features of PSVD

项目	门静脉高压表现	PSVD病理表现^1）
特征性	·胃、食管或异位静脉曲张 ·门静脉高压性出血 ·影像学发现门体侧支循环	·门静脉闭塞（血管壁增厚、管腔闭塞、门静脉分支消失） ·结节再生性增生 ·不完全间隔纤维化（也称为不完全间隔硬化）；该特征只能通过大体肝标本而非肝活检评估
非特征性	·腹水 ·PLT<150 000/mm³ ·脾脏最大长度≥13 cm	·门静脉支异常（动脉增殖、扩张，汇管区内与门静脉紧密相连的异常血管） ·血管结构紊乱：门静脉和中央静脉分布不规则 ·非带状肝窦扩张 ·轻度窦周纤维化
注：1）需由1名病理学专家明确。

下载: 导出CSV

参考文献(40)

[1]	HE FL, MA L, LI YR, et al. Non-cirrhotic portal hypertension: The highlight of its diagnosis[J]. J Pract Hepatol, 2022, 25( 1): 1- 4. DOI: 10.3969/j.issn.1672-5069.2022.01.001. 何福亮, 马琳, 李悦榕, 等. 非肝硬化性门脉高压的临床诊断[J]. 实用肝脏病杂志, 2022, 25( 1): 1- 4. DOI: 10.3969/j.issn.1672-5069.2022.01.001.
[2]	HERNÁNDEZ-GEA V, BAIGES A, TURON F, et al. Idiopathic portal hypertension[J]. Hepatology, 2018, 68( 6): 2413- 2423. DOI: 10.1002/hep.30132.
[3]	European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver[J]. J Hepatol, 2016, 64( 1): 179- 202. DOI: 10.1016/j.jhep.2015.07.040.
[4]	KHANNA R, SARIN SK. Noncirrhotic portal hypertension: Current and emerging perspectives[J]. Clin Liver Dis, 2019, 23( 4): 781- 807. DOI: 10.1016/j.cld.2019.07.006.
[5]	KMEID M, LIU XL, BALLENTINE S, et al. Idiopathic non-cirrhotic portal hypertension and porto-sinusoidal vascular disease: Review of current data[J]. Gastroenterology Res, 2021, 14( 2): 49- 65. DOI: 10.14740/gr1376.
[6]	KHANNA R, SARIN SK. Idiopathic portal hypertension and extrahepatic portal venous obstruction[J]. Hepatol Int, 2018, 12( Suppl 1): 148- 167. DOI: 10.1007/s12072-018-9844-3.
[7]	de GOTTARDI A, RAUTOU PE, SCHOUTEN J, et al. Porto-sinusoidal vascular disease: Proposal and description of a novel entity[J]. Lancet Gastroenterol Hepatol, 2019, 4( 5): 399- 411. DOI: 10.1016/S2468-1253(19)30047-0.
[8]	de FRANCHIS R, BOSCH J, GARCIA-TSAO G, et al. Baveno VII-Renewing consensus in portal hypertension[J]. J Hepatol, 2022, 76( 4): 959- 974. DOI: 10.1016/j.jhep.2021.12.022.
[9]	CAI ZH, ZHUGE YZ. Disease characteristics, diagnosis and treatment progress of vascular diseases of portal vein and hepatic sinus[J]. Chin Hepatol, 2023, 28( 2): 148- 151. DOI: 10.14000/j.cnki.issn.1008-1704.2023.02.007. 蔡子豪, 诸葛宇征. 门静脉肝窦血管性疾病的疾病特点与诊疗进展[J]. 肝脏, 2023, 28( 2): 148- 151. DOI: 10.14000/j.cnki.issn.1008-1704.2023.02.007.
[10]	NICOARĂ-FARCĂU O, RUSU I, STEFĂNESCU H, et al. Diagnostic challenges in non-cirrhotic portal hypertension-Porto sinusoidal vascular disease[J]. World J Gastroenterol, 2020, 26( 22): 3000- 3011. DOI: 10.3748/wjg.v26.i22.3000.
[11]	WÖRAN K, SEMMLER G, JACHS M, et al. Clinical course of porto-sinusoidal vascular disease is distinct from idiopathic noncirrhotic portal hypertension[J]. Clin Gastroenterol Hepatol, 2022, 20( 2): e251- e266. DOI: 10.1016/j.cgh.2020.11.039.
[12]	GUIDO M, ALVES VAF, BALABAUD C, et al. Histology of portal vascular changes associated with idiopathic non-cirrhotic portal hypertension: Nomenclature and definition[J]. Histopathology, 2019, 74( 2): 219- 226. DOI: 10.1111/his.13738.
[13]	RIGGIO O, GIOIA S, PENTASSUGLIO I, et al. Idiopathic noncirrhotic portal hypertension: Current perspectives[J]. Hepat Med, 2016, 8: 81- 88. DOI: 10.2147/HMER.S85544.
[14]	GIOIA S, NARDELLI S, PASQUALE C, et al. Natural history of patients with non cirrhotic portal hypertension: Comparison with patients with compensated cirrhosis[J]. Dig Liver Dis, 2018, 50( 8): 839- 844. DOI: 10.1016/j.dld.2018.01.132.
[15]	ZHANG HW, WANG ML. Progress in the clinical diagnosis of idiopathic noncirrhotic portal hypertension[J]. Chin J Hepatobiliary Surg, 2018, 24( 9): 636- 640. DOI: 10.3760/cma.j.issn.1007-8118.2018.09.017. 张宏伟, 王孟龙. 特发性非硬化性门静脉高压的临床诊断研究进展[J]. 中华肝胆外科杂志, 2018, 24( 9): 636- 640. DOI: 10.3760/cma.j.issn.1007-8118.2018.09.017.
[16]	HE FL, QI RZ, ZHANG K, et al. Curative effect analysis of splenectomy and esophagogastric devascularization on esophageal and gastric varices bleeding in patients with idiopathic noncirrhotic portal hypertension[J]. J Clin Exp Med, 2020, 19( 13): 1402- 1406. DOI: 10.3969/j.issn.1671-4695.2020.13.018. 何福亮, 齐瑞兆, 张珂, 等. 脾切除联合贲门周围血管离断术治疗特发性非硬化性门脉高压所致食管胃底静脉曲张破裂出血的疗效分析[J]. 临床和实验医学杂志, 2020, 19( 13): 1402- 1406. DOI: 10.3969/j.issn.1671-4695.2020.13.018.
[17]	PULVIRENTI F, PENTASSUGLIO I, MILITO C, et al. Idiopathic non cirrhotic portal hypertension and spleno-portal axis abnormalities in patients with severe primary antibody deficiencies[J]. J Immunol Res, 2014, 2014: 672458. DOI: 10.1155/2014/672458.
[18]	LEE H, REHMAN AU, FIEL MI. Idiopathic noncirrhotic portal hypertension: An appraisal[J]. J Pathol Transl Med, 2016, 50( 1): 17- 25. DOI: 10.4132/jptm.2015.09.23.
[19]	TSUNEYAMA K, HARADA K, KATAYANAGI K, et al. Overlap of idiopathic portal hypertension and scleroderma: Report of two autopsy cases and a review of literature[J]. J Gastroenterol Hepatol, 2002, 17( 2): 217- 223. DOI: 10.1046/j.1440-1746.2002.02587.x.
[20]	KOGAWA H, MIGITA K, ITO M, et al. Idiopathic portal hypertension associated with systemic sclerosis and Sjögren’s syndrome[J]. Clin Rheumatol, 2005, 24( 5): 544- 547. DOI: 10.1007/s10067-005-1084-y.
[21]	KOTANI K, KAWABE J, MORIKAWA H, et al. Comprehensive screening of gene function and networks by DNA microarray analysis in Japanese patients with idiopathic portal hypertension[J]. Mediators Inflamm, 2015, 2015: 349215. DOI: 10.1155/2015/349215.
[22]	GUIDO M, SARCOGNATO S, SACCHI D, et al. Pathology of idiopathic non-cirrhotic portal hypertension[J]. Virchows Arch, 2018, 473( 1): 23- 31. DOI: 10.1007/s00428-018-2355-8.
[23]	VISPO E, CEVIK M, ROCKSTROH JK, et al. Genetic determinants of idiopathic noncirrhotic portal hypertension in HIV-infected patients[J]. Clin Infect Dis, 2013, 56( 8): 1117- 1122. DOI: 10.1093/cid/cit001.
[24]	HILLAIRE S, BONTE E, DENNINGER MH, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: A re-evaluation in 28 patients[J]. Gut, 2002, 51( 2): 275- 280. DOI: 10.1136/gut.51.2.275.
[25]	SIRAMOLPIWAT S, SEIJO S, MIQUEL R, et al. Idiopathic portal hypertension: Natural history and long-term outcome[J]. Hepatology, 2014, 59( 6): 2276- 2285. DOI: 10.1002/hep.26904.
[26]	YE JY, XIE YL, XU YJ, et al. Case report: Oxaliplatin-induced idiopathic non-cirrhotic portal hypertension: A case report and literature review[J]. Front Med, 2023, 10: 1285064. DOI: 10.3389/fmed.2023.1285064.
[27]	SCHOUTEN JNL, VERHEIJ J, SEIJO S. Idiopathic non-cirrhotic portal hypertension: A review[J]. Orphanet J Rare Dis, 2015, 10: 67. DOI: 10.1186/s13023-015-0288-8.
[28]	BESMOND C, VALLA D, HUBERT L, et al. Mutations in the novel gene FOPV are associated with familial autosomal dominant and non-familial obliterative portal venopathy[J]. Liver Int, 2018, 38( 2): 358- 364. DOI: 10.1111/liv.13547.
[29]	JIN SJ, CHOI WM. Porto-sinusoidal vascular disease: A concise updated summary of epidemiology, pathophysiology, imaging, clinical features, and treatments[J]. Korean J Radiol, 2023, 24( 1): 31- 38. DOI: 10.3348/kjr.2022.0668.
[30]	KHANNA R, SARIN SK. Non-cirrhotic portal hypertension-diagnosis and management[J]. J Hepatol, 2014, 60( 2): 421- 441. DOI: 10.1016/j.jhep.2013.08.013.
[31]	ELKRIEF L, LAZARETH M, CHEVRET S, et al. Liver stiffness by transient elastography to detect porto-sinusoidal vascular liver disease with portal hypertension[J]. Hepatology, 2021, 74( 1): 364- 378. DOI: 10.1002/hep.31688.
[32]	MATSUTANI S, MARUYAMA H, AKIIKE T, et al. Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan[J]. Liver Int, 2005, 25( 5): 978- 983. DOI: 10.1111/j.1478-3231.2005.01151.x.
[33]	de GOTTARDI A, SEMPOUX C, BERZIGOTTI A. Porto-sinusoidal vascular disorder[J]. J Hepatol, 2022, 77( 4): 1124- 1135. DOI: 10.1016/j.jhep.2022.05.033.
[34]	WAGURI N, SUDA T, KAMURA T, et al. Heterogeneous hepatic enhancement on CT angiography in idiopathic portal hypertension[J]. Liver, 2002, 22( 3): 276- 280. DOI: 10.1046/j.0106-9543.2002.01663.x.
[35]	KANG JH, KIM DH, KIM SY, et al. Porto-sinusoidal vascular disease with portal hypertension versus liver cirrhosis: Differences in imaging features on CT and hepatobiliary contrast-enhanced MRI[J]. Abdom Radiol(NY), 2021, 46( 5): 1891- 1903. DOI: 10.1007/s00261-020-02831-w.
[36]	OSADA Y, KANAZAWA H, NARAHARA Y, et al. Wedged hepatic venous pressure does not reflect portal pressure in patients with cirrhosis and hepatic veno-venous communications[J]. Dig Dis Sci, 2008, 53( 1): 7- 13. DOI: 10.1007/s10620-007-0039-3.
[37]	BISSONNETTE J, GARCIA-PAGÁN JC, ALBILLOS A, et al. Role of the transjugular intrahepatic portosystemic shunt in the management of severe complications of portal hypertension in idiopathic noncirrhotic portal hypertension[J]. Hepatology, 2016, 64( 1): 224- 231. DOI: 10.1002/hep.28547.
[38]	GIOIA S, NARDELLI S, RIDOLA L, et al. Causes and management of non-cirrhotic portal hypertension[J]. Curr Gastroenterol Rep, 2020, 22( 12): 56. DOI: 10.1007/s11894-020-00792-0.
[39]	GIOIA S, RIGGIO O, NARDELLI S, et al. Identifying patients at high risk of developing non-cirrhotic portal hypertension[J]. Hepat Med, 2021, 13: 105- 111. DOI: 10.2147/HMER.S282674.
[40]	ZUO CL, CHUMBALKAR V, ELLS PF, et al. Prevalence of histological features of idiopathic noncirrhotic portal hypertension in general population: A retrospective study of incidental liver biopsies[J]. Hepatol Int, 2017, 11( 5): 452- 460. DOI: 10.1007/s12072-017-9801-6.