Pathology of the Liver in “Idiopathic Portal Hypertension” Associated with Autoimmune Disease

Nakanuma, Yasuni; Nonomura, Akitaka; Hayashi, Morimoto; Doishita, Kenji; Takayanagi, Nobutatsu; Uchida, Tatsuo; Obata, Yoko; Noma, K.; Ikoma, Jiro; Yoshikawa, Kunihiko

doi:10.1111/j.1440-1827.1989.tb02488.x

Cited by 21 publications

(17 citation statements)

References 9 publications

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“…All of the etiologies of NCPH result in increased resistance to portal blood flow at 1 of 3 levels; the impediment to blood flow can occur at the extrahepatic presinusoidal level (i.e., portal vein thrombosis), at the intrahepatic (presinusoidal, sinusoidal, or postsinusoidal level, Table 3) or at the extrahepatic postsinusoidal level (i.e., Budd‐Chiari syndrome) 18. Several different terms have been used to describe the clinicopathologic features of the intrahepatic disorders, including idiopathic portal hypertension,19–25 noncirrhotic portal fibrosis,14 NRH,1–6 ISC,7, 8 and hepatoportal sclerosis 9, 10. Regardless of the level of resistance, most patients develop gastric and esophageal varices; ascites and encephalopathy tend to develop in patients with sinusoidal or intrahepatic postsinusoidal disease 18.…”

Section: Discussionmentioning

confidence: 99%

Liver transplantation for severe intrahepatic noncirrhotic portal hypertension

et al. 2005

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Intrahepatic noncirrhotic portal hypertension can be idiopathic or associated with known toxic, developmental, vascular, or biliary tract diseases. Most patients are successfully managed medically or with shunting procedures. The goal of this study was to explore the reasons some patients require orthotopic liver transplantation (OLT). The clinical features, gross and microscopic liver explant pathology, and posttransplantation course in 16 patients who underwent OLT for intrahepatic noncirrhotic portal hypertension were studied. There were 11 men and 5 women with a mean age of 47 years. Clinical manifestations included gastrointestinal varices (n ‫؍‬ 12), ascites (n ‫؍‬ 8), encephalopathy (n ‫؍‬ 3), and hepatopulmonary syndrome (n ‫؍‬ 3). Cirrhosis was misdiagnosed clinically, radiographically and/or histologically in 13 patients (81%). Grossly, liver explants weighed a mean of 1,100 g, and 12 had a nodular appearance. Histologically, all 16 livers had portal tract vascular abnormalities, 15 had nodular regenerative hyperplasia (NRH), and 9 had incomplete septal cirrhosis. After OLT, mild NRH features were noted in 2 patients, and 1 of these patients developed evidence of portal hypertension. This study demonstrates that a subset of patients with intrahepatic noncirrhotic portal hypertension have severe symptoms requiring OLT. Accurate pre-OLT diagnosis is frequently difficult at advanced stages of the disease; 81% of our patients carried a diagnosis of cirrhosis. Morphologically, the explanted livers showed evidence of vascular abnormalities, NRH, and increased fibrosis, but not cirrhosis. Importantly, however, a diagnosis of cirrhosis is not required in this group of patients to qualify them for OLT, and these patients have good long-term graft function after OLT. (Liver Transpl 2005;11:627-634.)

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Section: Discussionmentioning

confidence: 99%

Liver transplantation for severe intrahepatic noncirrhotic portal hypertension

et al. 2005

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“…The portal vein and its branches are prominent and have sclerosed walls. Autopsy series commonly show thrombosis in the medium and small (diameter < 300 µm) portal vein branches 23,24 …”

Section: Pathologymentioning

confidence: 99%

Non‐cirrhotic portal fibrosis: Current concepts and management

Sarin

Kapoor

2002

J of Gastro and Hepatol

150

183

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Non-cirrhotic portal hypertension (NCPH) comprises diseases having an increase in portal pressure (PP) due to intraheptic or prehepatic lesions, in the absence of cirrhosis. The lesions are generally vascular, either in the portal vein, its branches or in the perisinusoidal area. Because the wedged hepatic venous pressure is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess PP. The majority of diseases included in the category of NCPH are well-characterized disease entities where portal hypertension (PHT) is a late manifestation and, hence, these are not discussed. Two diseases that present only with features of PHT and are common in developing countries are non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO). Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by 'obliterative portovenopathy' leading to PHT, massive splenomegaly and well-tolerated episodes of variceal bleeding in young adults from low socioeconomic backgrounds, having near normal hepatic functions. In some parts of the world, NCPF is called idiopathic portal hypertension (IPH) or 'hepatoportal sclerosis'. Because 85-95% of patients with NCPF and EHPVO present with variceal bleeding, treatment involves management with endoscopic sclerotherapy (EST) or variceal ligation (EVL). These therapies are effective in approximately 90-95% of patients. Gastric varices are another common cause of upper gastrointestinal bleeding in these patients and these can be managed with cyanoacrylate glue injection or surgery. Other indications for surgery include failure of EST/EVL, and symptomatic hypersplenism. The prognosis of patients with NCPF is good and 5 years survival in patients in whom variceal bleeding can be controlled has been reported to be approximately 95-100%.

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“…Evidence of previous phlebothrombosis is suggested by the presence of old mural thrombi incorporated in the wall, mural thickening of the extrahepatic portal vein and coexistence of lesions characteristic of NCPF as well as EHPVO in the same patient. Pathology of IPH differs from the NCPF due to the associated autoimmune conditions 12,31 …”

Section: Pathologymentioning

confidence: 99%

Non‐cirrhotic portal fibrosis

Sarin

2002

J of Gastro and Hepatol

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Non‐cirrhotic portal hypertension (NCPH) comprises of diseases having an increase in portal pressure (PP) due to intraheptic or prehepatic lesions, in the absence of cirrhosis. The lesions are generally vascular, either in the portal vein, its branches or in the perisinusoidal area. Because the wedged hepatic venous pressure (WHVP) is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess portal pressure. The majority of the diseases included in the category of NCPH are well characterized disease entities where portal hypertension (PHT) is a late manifestation and hence, these are not discussed. Two diseases which present only with features of PHT and are common in developing countries are NCPF and extra‐hepatic portal vein obstruction (EHPVO). Non‐cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by ‘Obliterative portovenopathy’ leading to PHT, massive splenomegaly, repeated well tolerated episodes of variceal bleeding and anemia in young adults from low socio‐economic strata of life. The hepatic parenchymal functions are nearly normal. Jaundice, ascites and hepatic encephalopathy are rare. Management of variceal bleeding remains the main concern as nearly 85% of patients with NCPF present with variceal bleeding. Endoscopic variceal ligation or sclerotherapy are equally effective in about 90–95% of the patients. Gastric varices are seen in about 25% patients and a bleed from them can be managed with cyanoacrylate glue injection or surgery. Other indications for surgery include failure of endoscopic therapy to control acute bleed and symptomatic hypersplenism. The prognosis of patients with NCPF is good and 5‐years survival rates in patients in whom variceal bleeding can be controlled is about > 95%. © 2002 Blackwell Publishing Asia Pty Ltd

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Pathology of the Liver in “Idiopathic Portal Hypertension” Associated with Autoimmune Disease

Cited by 21 publications

References 9 publications

Liver transplantation for severe intrahepatic noncirrhotic portal hypertension

Liver transplantation for severe intrahepatic noncirrhotic portal hypertension

Non‐cirrhotic portal fibrosis: Current concepts and management

Non‐cirrhotic portal fibrosis

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