Idiopathic portal hypertension associated with systemic sclerosis and Sjögren’s syndrome

Kogawa, Hiroyuki; Migita, Kiyoshi; Ito, Masahiro; Takii, Yasushi; Daikoku, Manabu; Nakao, M; Miyashita, Taichiro; Kimura, Hironori; Ezaki, Hironori; Nakamura, Minoru; Yatsuhashi, Hiroshi; Eguchi, Katsumi; Ishibashi, Hiromi

doi:10.1007/s10067-005-1084-y

Cited by 15 publications

(9 citation statements)

References 13 publications

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“…Multiple studies have reported an association between INCPH/ PSVD and disorders of immune function including congenital [85] (in particular common variable immunodeficiency (CVID)) and acquired immunodeficiencies (human immunodeficiency virus (HIV)-related) as well as autoimmune diseases [2,10]. For example, INCPH has been described in patients with inflammatory bowel diseases, rheumatoid arthritis, systemic lupus erythematosus, autoimmune hepatitis, scleroderma, Felty's syndrome and celiac disease among others [10,24,28,29,40,[63][64][65]86]. Seropositivity for antinuclear, antimicrosomal and anti-DNA antibodies has also been reported [3,23].…”

Section: Immune Dysregulationmentioning

confidence: 99%

Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data

et al. 2021

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Idiopathic non-cirrhotic portal hypertension (INCPH) is a clinicopathologic disease entity characterized by the presence of clinical signs and symptoms of portal hypertension (PH) in the absence of liver cirrhosis or known risk factors accountable for PH. Multiple hematologic, immune-related, infectious, hereditary and metabolic risk factors have been associated with this disorder. Still, the exact etiopathogenesis is largely unknown. The recently proposed portosinusoidal vascular disease (PSVD) scheme broadens the spectrum of the disease by also including patients without clinical PH who are found to have similar histopathologic findings on core liver biopsies. Three histomorphologic lesions have been identified as specific for PSVD to include obliterative portal venopathy, nodular regenerative hyperplasia and incomplete septal cirrhosis/fibrosis. However, these findings are often subtle, under-recognized and subjective with low interobserver agreement among pathologists. Additionally, the natural history of the subclinical forms of the disease remains unexplored. The clinical course is more favorable compared to cirrhosis patients, especially in the absence of clinical PH or liver dysfunction. There are no universally accepted guidelines in regard to diagnosis and treatment of INCPH/PSVD. Hence, this review emphasizes the need to raise awareness of this entity by highlighting its complex pathophysiology and clinicopathologic associations. Lastly, formulation of standardized diagnostic criteria with clinical validation is necessary to avoid misclassifying vascular diseases of the liver and to develop and implement targeted therapeutic strategies.

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Section: Immune Dysregulationmentioning

confidence: 99%

Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data

et al. 2021

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“…Higher prevalence of INCPH in lower socioeconomic groups and experimental animal studies indicate an infectious etiology [ 3 , 52 , 58 , 59 ]. Especially in the West, INCPH is increasingly recognized in human immunodeficiency virus (HIV) patients [ 3 , 32 ].…”

Section: Etiologymentioning

confidence: 99%

Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

Lee¹,

Rehman

Fiel

2016

J Pathol Transl Med

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Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity.

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“…[7][8][9][10] The etiology of IPH is still not clear. 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).…”

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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Idiopathic portal hypertension associated with systemic sclerosis and Sjögren’s syndrome

Cited by 15 publications

References 13 publications

Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data

Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data

Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

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

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