Histopathological, Clinical and Epidemiological Features of Hepatoportal Sclerosis in a Referral Center for Liver Disease in Northeastern Brazil

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 histopatological similarities with the hepatosplenic form of schistosomiasis. OBJECTIVE: To analyze the profile of patients with HPS in Northeastern Brazil and to demonstrate the pathological characteristics of HPS. METHODS: We retrospectively analyzed cases of OPV in liver biopsies and explants from a referral center for liver in Bahia - Brazil. The qualitative and quantitative analysis of the portal tracts and liver parenchyma was made so that comparisons could be done among the HPS findings of our population and the findings described by other authors. RESULTS: From the 62 patients identified with HPS, 42% were male, while 58% were female. The average age at diagnosis was 48.3 years. From this group, we analyzed the liver biopsy of 10 patients whose diagnosis of schistosomiasis could be ruled out. From these 100% (10/10) presented dense portal fibrosis and portal venous obliteration. Liver parenchymal atrophy was present in 60% (6/10) of the patients, sinusoidal dilation was present in 30% (3/10), the presence of portal septa occurred in 50% (5/10) and dense portal fibrosis in all patients analyzed. Nodular regenerative hyperplasia was found in 30% (3/10) of the patients. CONCLUSION: HPS seems to be neglected and misdiagnosed in Brazil, due to its similarities with schistossomiasis. In our study dense portal fibrosis, obliteration of the portal vein branches, parenchymal atrophy, sinusoidal dilatation and parenchymal nodular hyperplasia were the main histopathological findings and were similar to that described in other countries.

Obliterative portal venopathy: A neglected and probably misdiagnosed disease with peculiar etiology in South America

“…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 …”

“…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.…”

“…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.…”

“…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 …”

“…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 …”