Histological Spectrum of Idiopathic Noncirrhotic Portal Hypertension in Liver Biopsies From Dialysis Patients

Lee, Hwajeong; Ainechi, Sanaz; Singh, Mandeep; Ells, Peter; Sheehan, Christine E.; Ji, Lin

doi:10.1177/1066896915582264

Cited by 14 publications

(20 citation statements)

References 35 publications

(80 reference statements)

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“…Some of the proposed etiological factors have included immunologic disorders, [11][12][13][14][15][16] chronic infection with some bacteria or human immunodeficiency virus, 13,[17][18][19] medication and toxins, [20][21][22][23][24] gene mutation, [25][26][27][28][29] and some other etiologies like dialysis. 30 The main clinical symptoms of IPH are those caused by portal hypertension, including splenomegaly, hypersplenism, hepatomegaly, gastrointestinal bleeding, ascites, and portal vein thrombosis (PVT). However, no specific symptoms of IPH have been found.…”

Section: Introductionmentioning

confidence: 99%

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Sun

Shao

et al. 2018

J of Gastro and Hepatol

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Background and Aim Idiopathic portal hypertension (IPH) refers to a relatively rare condition characterized by intrahepatic portal hypertension in the absence of underlying disease such as liver cirrhosis. Methods We retrospectively reviewed 338 patients with IPH that were diagnosed at the pathological consultation center of our hospital. Results The ratio of male to female patients was 1:1. Mean age at onset was 35.1 ± 16.5 years; male patients on average were 12 years younger than female patients at onset. The median duration from onset to IPH diagnosis was 12 months. In 50 patients, medication use may have been an etiological factor. The most common clinical manifestations were splenomegaly (91.3%) and hypersplenism (68.9%); 57.0% patients presented varicosis, while 25.1% patients had a history of variceal bleeding. Nodular regenerative hyperplasia was found in 22.2% liver biopsies. Among patients for whom laboratory data were available, 65.0%, 50.3%, and 71.4% patients presented leukopenia, anemia, and thrombocytopenia due to hypersplenism. Liver function was mostly in the compensated stage. Female patients showed worse leukopenia and anemia, while male patients were more likely to have abnormal serum transaminase and bilirubin levels. Sixty‐seven patients received surgical or interventional treatment. Conclusions High‐quality liver biopsy, detailed clinical information, and expert pathologist are necessary for diagnosis of IPH. IPH can occur concurrently with other liver disease such as hepatitis and drug‐induced liver injury. Medication appears to be an important etiological factor for IPH in China. Management approach was largely focused on treatment of portal hypertension and its complications.

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Section: Introductionmentioning

confidence: 99%

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Sun

Shao

et al. 2018

J of Gastro and Hepatol

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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].…”

Section: Introductionmentioning

confidence: 99%

“…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%

“…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. Therefore, as previously devised and utilized [10,11], the individual histologic lesions of PSVD were evaluated for the absence (score 0) or presence (score 1) of the lesions, and the combined score (CS) of the individual lesions was assessed.…”

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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“…Pathologic studies over many years have clarified the characteristics of various pathologic changes in the INCPH liver [1][2][3][4][5][6][7][8][9]. They can be briefly summarized as follows.…”

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confidence: 99%

Pathology of idiopathic non-cirrhotic portal hypertension

Kage

2017

Hepatol Int

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Histological Spectrum of Idiopathic Noncirrhotic Portal Hypertension in Liver Biopsies From Dialysis Patients

Abstract: Liver biopsies from patients on dialysis demonstrate histopathological vascular changes of INCPH. Some (31%) patients present with portal hypertension without cirrhosis. The histological changes may be reflective of underlying risk factors for INCPH in this group.

Cited by 14 publications

References 35 publications

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

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

Pathology of idiopathic non-cirrhotic portal hypertension

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