Abstract:BACKGROUND: Hepatoportal sclerosis HPS or obliterative portal venopathy (OPV), one of the differential diagnoses for non-cirrohtic portal hypertension, is characterized by the disappearance of the portal branches, portal and septal fibrosis, perisinusoidal fibrosis and regenerative nodular hyperplasia (RNH). It is a spectral disease that may progress to severe portal hypertension. Its etiopathogenesis is still little understood, especially in Brazil, it has been probably misdiagnosed due to its histopatologic… Show more
“…In addition, our liver unit has frequently diagnosed cases of OPV, mainly in patients with persistent abnormal liver enzymes. Our analysis further leads us to believe that patients with OPV could have been misdiagnosed with schistosomiasis in the recent past 4 …”
Section: Discussionmentioning
confidence: 79%
“…Our analysis further leads us to believe that patients with OPV could have been misdiagnosed with schistosomiasis in the recent past. 4 While the causes underlying OPV remain unclear, some authors have described strong associations with procoagulant and immunological conditions, as well as genetic predisposition. 23 Another possible association with drugs has been described in several case reports.…”
Section: Discussionmentioning
confidence: 99%
“…Additional findings may include hypertrophy of the intrahepatic artery branches, anomalous lymphatic and/or venous vessels with telangiectatic appearance, paraportal herniated vessels, sinusoidal dilation and/or perisinusoidal fibrosis, and nodular regenerative hyperplasia of the hepatocytes. [2][3][4] In northeastern Brazil, schistosomiasis is endemic; the hepatosplenic form remains a common cause of non-cirrhotic portal hypertension. Accordingly, cases of OPV may be misdiagnosed as portal hypertension associated with schistosomiasis.…”
Section: Introductionmentioning
confidence: 99%
“…The most characteristic histopathologic finding is a fibrous enlargement of the portal tracts associated with either the disappearance or the reduction in the caliber of the portal vein branches in the absence of cirrhosis. Additional findings may include hypertrophy of the intrahepatic artery branches, anomalous lymphatic and/or venous vessels with telangiectatic appearance, paraportal herniated vessels, sinusoidal dilation and/or perisinusoidal fibrosis, and nodular regenerative hyperplasia of the hepatocytes 2–4 …”
Section: Introductionmentioning
confidence: 99%
“…Additional findings may include hypertrophy of the intrahepatic artery branches, anomalous lymphatic and/or venous vessels with telangiectatic appearance, paraportal herniated vessels, sinusoidal dilation and/or perisinusoidal fibrosis, and nodular regenerative hyperplasia of the hepatocytes. 2 , 3 , 4 …”
Background and Aim
Obliterative portal venopathy (OPV) is one of the causes of non‐cirrhotic portal hypertension. However, many aspects of OPV remain unclear, including the etiology, pathogenesis, and natural history. The aim of this study was to describe the clinical features of OPV in a series of patients in Brazil in whom OPV was diagnosed through liver biopsy.
Methods
Forty‐three consecutive adult patients with OPV were retrospectively selected as a case series based on histologic criteria, defined by the presence of at least portal fibrosis, phlebosclerosis, disappearance and/or reduction of the caliber of portal vein branches, and exclusion of cirrhosis. Clinical and laboratory data were analyzed. Clinically significant portal hypertension was considered in the presence of esophageal varices and/or ascites.
Results
The mean age of patients at diagnosis was 44.5 ± 11 years, who were predominantly female (81%). Clinically significant portal hypertension was found in 28% of cases. The most frequent indication for liver biopsy was the elevation of liver enzymes, mostly γ‐glutamyl transferase (GGT) in 76% of patients, averaging 222 IU/L (upper limit of normality up to 40 IU/L) and alanine aminotransferase (ALT) in 64%, mean 84 IU/L (38 IU/L). One‐third of our patients had exposure to medications, especially herbal medicines, at the time of enzymatic changes. Other risk factors highlighted were features of autoimmunity in 25% of patients or thrombophilia in 20%.
Conclusion
OPV can be diagnosed even before the onset of portal hypertension, ALT elevation, and especially GGT elevation in most cases. Its etiology is not defined, but autoimmune diseases, thrombophilia, and the use of medications or herbal medicines may play a role.
“…In addition, our liver unit has frequently diagnosed cases of OPV, mainly in patients with persistent abnormal liver enzymes. Our analysis further leads us to believe that patients with OPV could have been misdiagnosed with schistosomiasis in the recent past 4 …”
Section: Discussionmentioning
confidence: 79%
“…Our analysis further leads us to believe that patients with OPV could have been misdiagnosed with schistosomiasis in the recent past. 4 While the causes underlying OPV remain unclear, some authors have described strong associations with procoagulant and immunological conditions, as well as genetic predisposition. 23 Another possible association with drugs has been described in several case reports.…”
Section: Discussionmentioning
confidence: 99%
“…Additional findings may include hypertrophy of the intrahepatic artery branches, anomalous lymphatic and/or venous vessels with telangiectatic appearance, paraportal herniated vessels, sinusoidal dilation and/or perisinusoidal fibrosis, and nodular regenerative hyperplasia of the hepatocytes. [2][3][4] In northeastern Brazil, schistosomiasis is endemic; the hepatosplenic form remains a common cause of non-cirrhotic portal hypertension. Accordingly, cases of OPV may be misdiagnosed as portal hypertension associated with schistosomiasis.…”
Section: Introductionmentioning
confidence: 99%
“…The most characteristic histopathologic finding is a fibrous enlargement of the portal tracts associated with either the disappearance or the reduction in the caliber of the portal vein branches in the absence of cirrhosis. Additional findings may include hypertrophy of the intrahepatic artery branches, anomalous lymphatic and/or venous vessels with telangiectatic appearance, paraportal herniated vessels, sinusoidal dilation and/or perisinusoidal fibrosis, and nodular regenerative hyperplasia of the hepatocytes 2–4 …”
Section: Introductionmentioning
confidence: 99%
“…Additional findings may include hypertrophy of the intrahepatic artery branches, anomalous lymphatic and/or venous vessels with telangiectatic appearance, paraportal herniated vessels, sinusoidal dilation and/or perisinusoidal fibrosis, and nodular regenerative hyperplasia of the hepatocytes. 2 , 3 , 4 …”
Background and Aim
Obliterative portal venopathy (OPV) is one of the causes of non‐cirrhotic portal hypertension. However, many aspects of OPV remain unclear, including the etiology, pathogenesis, and natural history. The aim of this study was to describe the clinical features of OPV in a series of patients in Brazil in whom OPV was diagnosed through liver biopsy.
Methods
Forty‐three consecutive adult patients with OPV were retrospectively selected as a case series based on histologic criteria, defined by the presence of at least portal fibrosis, phlebosclerosis, disappearance and/or reduction of the caliber of portal vein branches, and exclusion of cirrhosis. Clinical and laboratory data were analyzed. Clinically significant portal hypertension was considered in the presence of esophageal varices and/or ascites.
Results
The mean age of patients at diagnosis was 44.5 ± 11 years, who were predominantly female (81%). Clinically significant portal hypertension was found in 28% of cases. The most frequent indication for liver biopsy was the elevation of liver enzymes, mostly γ‐glutamyl transferase (GGT) in 76% of patients, averaging 222 IU/L (upper limit of normality up to 40 IU/L) and alanine aminotransferase (ALT) in 64%, mean 84 IU/L (38 IU/L). One‐third of our patients had exposure to medications, especially herbal medicines, at the time of enzymatic changes. Other risk factors highlighted were features of autoimmunity in 25% of patients or thrombophilia in 20%.
Conclusion
OPV can be diagnosed even before the onset of portal hypertension, ALT elevation, and especially GGT elevation in most cases. Its etiology is not defined, but autoimmune diseases, thrombophilia, and the use of medications or herbal medicines may play a role.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.