Autoimmune biomarkers in porto‐sinusoidal vascular disease: Potential role in its diagnosis and pathophysiology

Reyes, Eira Cerda; González-Navarro, Europa Azucena; Magaz, Marta; Muñoz-Sánchez, Guillermo; Díaz, Alba; Silva‐Junior, Gilberto; Triguero, Ana; Lafoz, Erica; Campreciós, Genís; Orts, Lara; Pérez-Campuzano, Valeria; Seijó, Susana; Rubió, Laura; Turón, Fanny; Baiges, Anna; Hernández‐Gea, Virginia; Alvarez‐Larrán, Alberto; Juan, Manel; García‐Pagán, Juan Carlos

doi:10.1111/liv.14997

Cited by 14 publications

(7 citation statements)

References 43 publications

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“…The pathophysiology of PSVD is poorly understood, 48 and therefore, there is a potential risk for PSVD recurrence after LT. However, recurrence of PSVD after LT had not been well studied.…”

Section: Discussionmentioning

confidence: 99%

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

et al. 2023

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Background. Porto-sinusoidal vascular liver disorder (PSVD) is a rare disease that occasionally requires liver transplantation (LT), despite usually presenting preserved liver function. There remains a paucity of data pertaining to LT in PSVD. The aim was to identify features associated with post-LT outcomes in PSVD. Methods. Retrospective multicentre study of 79 patients who received LT for PSVD. Results. Median post-LT follow-up was 37 (range 1-261) mo. Refractory ascites 24 (30%), hepatic encephalopathy 16 (20%), and hepatopulmonary syndrome 13 (16.3%) were the most frequent indications for LT. Hepatocellular carcinoma was the indication in only 2 patients. Twenty-four patients died, 7 due to liver and 17 to non-liver related causes. Post-LT survival was 82.2%, 80.7%, and 68.6% at 1, 2, and 5 y, respectively. Post-LT survival was significantly better in patients without (n = 58) than in those with a persistent severe PSVD-associated condition (n = 21). Pre-LT hyperbilirubinemia levels and creatinine >100 µmol/L were also independently associated with poor survival. Six patients (7.6%) required a second LT. Recurrence of PSVD was confirmed by liver biopsy in only 1 patient and in 3 further patients it was likely. Conclusions. LT in PSVD is associated with an acceptable outcome in the absence of associated severe conditions. However, persistence of a severe associated condition, pre-LT high bilirubin levels, or creatinine >100 µmol/L impact outcome, and these are features that should be considered when evaluating PSVD patients for LT. PSVD recurrence is possible after LT and needs to be explored, at least, in cases of posttransplant portal hypertension.

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“…The pathophysiology of PSVD is poorly understood, 48 and therefore, there is a potential risk for PSVD recurrence after LT. However, recurrence of PSVD after LT had not been well studied.…”

Section: Discussionmentioning

confidence: 99%

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

et al. 2023

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“…PSVD alters the portal vasculature and raise portal pressure irrespective of parenchymal liver disease and represents a unique It has been hypothesized that PSVD may originate from immune and inflammatory mechanisms that induce the activation of LSEC and the onset of local inflammation, leading to damage of small portal vein branches, endothelial dysfunction and activation of HSC, which in turn lead to further endothelial damage, phlebosclerosis, the onset of PH and finally apoptosis of liver cells. [62][63][64][65][66][67] This hypothesis suggests a central and exclusive role of LSEC in the pathophysiology of PSVD and onset of PH. However, it does not explain why only some patients with PSVD histological lesions develop PH, or why only some patients exposed to PSVD-associated factors develop the disease, while others with the same features do not.…”

Section: R Are G Ene Ti C C Aus E S Of Non -Cirrhotic P Ortal Hyperte...mentioning

confidence: 95%

“…It has been hypothesized that PSVD may originate from immune and inflammatory mechanisms that induce the activation of LSEC and the onset of local inflammation, leading to damage of small portal vein branches, endothelial dysfunction and activation of HSC, which in turn lead to further endothelial damage, phlebosclerosis, the onset of PH and finally apoptosis of liver cells 62–67 . This hypothesis suggests a central and exclusive role of LSEC in the pathophysiology of PSVD and onset of PH.…”

Section: Rare Genetic Causes Of Non‐cirrhotic Portal Hypertension a P...mentioning

confidence: 99%

The genetics of portal hypertension: Recent developments and the road ahead

Shalaby,

Ronzoni,

Hernandez‐Gea

et al. 2023

Liver International

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Portal hypertension (PH), defined as a pathological increase in the portal vein pressure, has different aetiologies and causes. Intrahepatic PH is mostly secondary to the presence of underlying liver disease leading to cirrhosis, characterized by parenchymal changes with deregulated accumulation of extracellular matrix and vascular abnormalities; liver sinusoidal endothelial cells and hepatic stellate cells are key players in PH progression, able to influence each other. However, PH may also develop independently of parenchymal damage, as occur in portosinusoidal vascular disorder (PSVD), a group of clinical and histological entities characterized by portal vasculature dysfunctions. In this particular group of disorders, the pathophysiology of PH is still poorly understood. In the last years, several genetic studies, based on genome‐wide association studies or whole‐exome sequencing analysis, have highlighted the importance of genetic heritability in PH pathogenesis, both in cirrhotic and non‐cirrhotic cases. The common PNPLA3 p.I148M variant, one of the main determinants of the susceptibility to steatotic liver disease, has also been associated with decompensation in patients with PH. Genetic variations at loci influencing coagulation, mainly the ABO locus, may directly contribute to the pathogenesis of PH. Rare genetic variants have been associated with familiar cases of progressive PSVD. In this review, we summarize the recent knowledges on genetic variants predisposing to PH development, contributing to better understand the role of genetic factors in PH pathogenesis.

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“…Recently, anti-endothelial cell antibodies (AECA) have been proposed as a marker for PSVD, but they are also positive in 16% of patients with cirrhosis [ 43 ]. Another potential biomarker is the activity of ADAMTS13 protease, whose activity is reduced in PSVD [ 44 ].…”

Section: Diagnosis Of Porto-sinusoidal Vascular Diseasementioning

confidence: 99%

Porto-sinusoidal vascular disease: a new definition of an old clinical entity

Barisic-Jaman,

Milosevic,

Pastrovic

et al. 2023

ceh

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Porto-sinusoidal vascular disease (PSVD) is defined as a vascular liver disease characterized by the absence of cirrhosis and the presence of characteristic histological features, with or without the presence of portal hypertension (PH). Half of the patients with PSVD also have associated disease that may contribute to the development of PSVD. Patients usually remain asymptomatic until complications of PH arise. Variceal bleeding and portal vein thrombosis are major complications associated with PSVD. The treatment is focused on managing complications of PH, mainly through primary prophylaxis of variceal bleeding and treatment of portal vein thrombosis. Currently, there is insufficient evidence to support the use of anticoagulants for thrombosis prevention in these patients. Despite the increase of recognition of PSVD, further research is needed to enable early disease diagnosis, establish optimal screening methods, and develop strategies to slow down disease progression.

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Autoimmune biomarkers in porto‐sinusoidal vascular disease: Potential role in its diagnosis and pathophysiology

Cited by 14 publications

References 43 publications

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

The genetics of portal hypertension: Recent developments and the road ahead

Porto-sinusoidal vascular disease: a new definition of an old clinical entity

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