Porto-sinusoidal vascular disease: proposal and description of a novel entity

Gottardi, Andrea De; Rautou, Pierre‐Emmanuel; Schouten, Jeoffrey N.L.; Rubbia‐Brandt, Laura; Leebeek, Frank; Trebicka, Jonel; Murad, Sarwa Darwish; Vilgrain, Valérie; Hernández‐Gea, Virginia; Nery, Filipe; Plessier, Aurélie; Berzigotti, Annalisa; Bioulac‐Sage, Paulette; Primignani, Massimo; Semela, David; Elkrief, Laure; Bédossa, Pierre; Valla, Dominique; García‐Pagán, Juan Carlos

doi:10.1016/s2468-1253(19)30047-0

Cited by 209 publications

(334 citation statements)

References 83 publications

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“…All patients had at least one clinical sign of portal hypertension (splenomegaly or hypersplenism, non-malignant ascites, minimally increased hepatic venous pressure gradient or portal-systemic collaterals). The patients did not have chronic liver diseases causing cirrhosis or non-cirrhotic portal hypertension, and they did not have congenital liver fibrosis, sarcoidosis or schistosomiasis, which cause non-cirrhotic portal hypertension 19 . The patients had patent portal and hepatic veins in Doppler ultrasound or CT scanning.…”

Section: Methodsmentioning

confidence: 99%

Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

Zhao

Wei

Wang

et al. 2020

Sci Rep

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Idiopathic portal hypertension (IPH) mimics liver cirrhosis in many aspects, and no efficient imaging method to differentiate the two diseases has been reported to date. In this study, the imaging and pathological characteristics were analysed for both IPH and cirrhosis. From January 2015 to March 2019, ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) images and pathological results from 16 IPH and 16 liver cirrhosis patients, as well as imaging results of 16 normal patients as a control group, were retrospectively reviewed. The age of the patients was 39 ± 20 years. There was a significant difference in the mean lumen diameter, wall thickness and ratio of thickness to diameter between the IPH and liver cirrhosis patients in the main and sagittal portal veins (P < 0.05), as well as in the lumen diameter and ratio of thickness to diameter between the IPH and liver cirrhosis patients in the Segment 3 (S3) portal vein (P < 0.05). In IPH patients, the main imaging changes were portal vein wall thickening, stenosis or occlusion, a low enhancement area along the portal vein in the delay phase in contrast-enhanced imaging, and a non-homogeneous change in T1WI. The corresponding pathological changes included interlobular vein thickening, stenosis, occlusion, portal area fibrosis, and atrophy or apoptosis of hepatocytes. The main imaging characteristic of liver cirrhosis was a nodular change in T1WI, and the related pathological change was pseudolobule formation. The imaging characteristics of IPH include thickening of the portal vein vascular wall, stenosis of the portal vein lumen and the absence of diffuse cirrhosis-like nodules. These imaging features have a definite pathological basis and could help make differential diagnoses between IPH and cirrhosis.

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Section: Methodsmentioning

confidence: 99%

Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

Zhao

Wei

Wang

et al. 2020

Sci Rep

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“…In this setting, the term portosinusoidal vascular diseases has been suggested as an overarching term for the heterogeneous clinical and histological features that can be seen with this form of liver disease. (2) Hepatic sinusoidal dilation describes the histological finding of widening hepatic capillaries that may predominate in the central, periportal, or medial areas or involve the entire lobule. This phenomenon can occur in a variety of situations, including in the setting of heart failure or hepatic venous outflow obstruction, in the vicinity of hepatic tumors, and, in cases of microvascular injury, to the sinusoids.…”

Section: Discussionmentioning

confidence: 99%

Oral Contraceptive–Induced Hepatic Sinusoidal Dilatation and Potential Implications for Living Donor Liver Transplantation: A Reason for Nonuse of Right Lobe Grafts

Stotts¹,

Wilkinson

Goldberg³

et al. 2020

Liver Transpl

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“…Idiopathic non-cirrhotic portal hypertension (INCPH) is a clinicopathologic entity in which liver samples from patients with clinical evidence of portal hypertension fail to show cirrhosis in the absence of other causes of portal hypertension [9][10][11][12][13]. Recently, the Vascular Liver Disease Interest Group (VALDIG) proposed a novel terminology "porto-sinusoidal vascular disease (PSVD)" in order to broaden the definition of INCPH and capture the earlier phase of the disease preceding portal hypertension [14]. Histologically, PSVD shows subtle vascular changes and associated histologic findings such as obliterative portal venopathy, nodular regenerative hyperplasia, incomplete septal fibrosis, portal tract abnormalities, architectural disturbance, non-zonal sinusoidal dilatation and mild perisinusoidal fibrosis, in the absence of cirrhosis [14].…”

Section: Introductionmentioning

confidence: 99%

“…Recently, the Vascular Liver Disease Interest Group (VALDIG) proposed a novel terminology "porto-sinusoidal vascular disease (PSVD)" in order to broaden the definition of INCPH and capture the earlier phase of the disease preceding portal hypertension [14]. Histologically, PSVD shows subtle vascular changes and associated histologic findings such as obliterative portal venopathy, nodular regenerative hyperplasia, incomplete septal fibrosis, portal tract abnormalities, architectural disturbance, non-zonal sinusoidal dilatation and mild perisinusoidal fibrosis, in the absence of cirrhosis [14]. Common risk factors include infectious etiology, hypercoagulability, rheumatologic and immunologic conditions, exposure to toxin/drug and genetic predisposition [9][10][11][12][13][14].…”

Section: Introductionmentioning

confidence: 99%

“…Unfortunately, there is no standardized method to quantify the degree or amount of individual histologic lesions pertaining to PSVD that correlates with clinical signs of portal hypertension [14,19]. Also, it is unknown how many of these individual histologic features or any combination thereof, is required to make a histologic diagnosis of PSVD or predict portal hypertension.…”

Section: Introductionmentioning

confidence: 99%

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Histologic Lesions of Porto-Sinusoidal Vascular Disease Following Phlebotomy in Hemochromatosis

et al. 2020

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Background: Phlebotomy induces regression of liver fibrosis in genetic hemochromatosis. We assessed the histologic changes in prephlebotomy and post-phlebotomy liver biopsies from patients with HFE mutation as a model to study regression of fibrosis. We aimed to show that phlebotomy-induced histologic lesions overlap with portosinusoidal vascular disease (PSVD, also known as idiopathic noncirrhotic portal hypertension), histologically. Methods: A total of 51 biopsies (22 pre-phlebotomy and 29 postphlebotomy) were reviewed, and three variables were studied: iron index indicative of the amount of accumulated iron (range 0 to 18), the combined score of vascular changes reflecting the presence of histological lesions that are described in PSVD (range 0 to 9) and the high-grade shunt vessel by calculating the proportion of portal tracts with shunt vessels, with a cutoff of 50%. Two-tailed Student's t-test and Fisher's exact test were performed to compare the means of two variables and frequencies of the histologic lesions in two groups, respectively. A P-value < 0.05 was considered statistically significant. Results: The iron index was higher in the pre-phlebotomy compared to post-phlebotomy group (P = 0.01). Compared to the pre-phlebotomy group, the combined score was higher in the post-phlebotomy group when the cases of advanced fibrosis were excluded (P = 0.023) and remained higher when patients with risk factors for PSVD were further excluded (P = 0.034). The high-grade shunt vessel tended to be more common in the post-phlebotomy group when advanced fibrosis was excluded; however, the statistical significance was marginal (P = 0.056). Conclusions: Phlebotomy reduces hepatic iron load and induces histologic lesions of PSVD in patients with HFE mutation. Our data support a postulation that some of the histologic lesions of PSVD represent vascular remodeling following a regression of fibrosis and may not be reflective of risk factors or etiopathogenesis of PSVD. Regressed fibrosis and PSVD may not be reliably distinguished in a limited sample, therefore warranting cautious interpretation in the right clinical context.

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Porto-sinusoidal vascular disease: proposal and description of a novel entity

Cited by 209 publications

References 83 publications

Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

Oral Contraceptive–Induced Hepatic Sinusoidal Dilatation and Potential Implications for Living Donor Liver Transplantation: A Reason for Nonuse of Right Lobe Grafts

Histologic Lesions of Porto-Sinusoidal Vascular Disease Following Phlebotomy in Hemochromatosis

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