Non-cirrhotic portal fibrosis at a tertiary care centre in South India

Venkatesan, Manigandan; Panicker, Lakshmi C; Sivamani, Kalaimani; Goel, Amit; Basu, Debdutta; Dhanapathi, Halnayak

doi:10.1177/0049475516636982

Cited by 9 publications

(7 citation statements)

References 13 publications

(21 reference statements)

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“…Previously, different terminologies have been used for INCPH/PSVD. Idiopathic portal hypertension [ 19 ] and non-cirrhotic portal fibrosis [ 20 ] were commonly used in Eastern countries (Japan and India, respectively), while the terms “OPV” and “hepatoportal sclerosis” were endorsed in the Western literature [ 10 ]. Other terminologies that have been used for this entity include “benign intrahepatic portal hypertension”, “intrahepatic noncirrhotic portal hypertension”, “noncirrhotic portal hypertension”, “idiopathic noncirrhotic intrahepatic portal hypertension”, “partial nodular transformation”, “incomplete septal cirrhosis” and “nodular regenerative hyperplasia” [ 1 - 3 , 6 , 7 , 10 , 21 ].…”

Section: Nomenclaturementioning

confidence: 99%

“…However, due to increased awareness of the disease and changes in national health policy, the incidence of INCPH has dramatically decreased in Japan to a total number of 11 new cases per year [ 2 ]. The high incidence of INCPH in India appears to be related to less favorable socio-economic conditions [ 20 , 80 ]. In North America and Europe, INCPH is considered a rare disease as it accounts for around 3-5% of PH cases [ 10 ].…”

Section: Epidemiologymentioning

confidence: 99%

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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: Nomenclaturementioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

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

et al. 2021

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“…In INCPH, the portal venous radicles show variable degrees of luminal narrowing, disappearance, or sclerosis . This change usually occurs in a fibrotic PT.…”

Section: Portal and Periportal Changes In Incphmentioning

confidence: 99%

“…A variety of terms were introduced for this non‐cirrhotic liver disease, such as Banti syndrome, non‐cirrhotic portal fibrosis, hepatoportal sclerosis, incomplete septal cirrhosis, obliterative portal venopathy, and nodular regenerative hyperplasia. After the recognition that these names appear to reflect a disease entity, albeit with heterogeneous morphological aspects, the umbrella term INCPH was introduced to standardise the nomenclature …”

Section: Introductionmentioning

confidence: 99%

“…The lack of pathognomonic clinical and laboratory findings makes the histological examination of liver tissue samples critically important to achieve a definite diagnosis of INCPH . INCPH liver may show a variety of lesions . The most characteristic encompass a constellation of portal/periportal vascular changes, supposedly involved in the pathogenesis.…”

Section: Introductionmentioning

confidence: 99%

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Histology of portal vascular changes associated with idiopathic non‐cirrhotic portal hypertension: nomenclature and definition

et al. 2018

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Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare vascular liver disease that has attracted new interest in recent years. It is characterised by clinical signs of portal hypertension in the absence of cirrhosis or severe fibrosis and any known cause of portal hypertension. As much uncertainty exists about INCPH pathophysiology, and no definite diagnostic tests are available, liver biopsy is an essential tool for achieving a definite diagnosis. Unfortunately, the histological diagnosis of INCPH is not always straightforward, as the characteristic lesions are unevenly distributed, vary greatly in their severity, are often very subtle, and are not all necessarily present in a single case. Furthermore, specifically for the characteristic portal vessel changes observed in INCPH, the terminology and definition are ambiguous, which adds complexity to the already complex clinicopathological scenario. An international study group of liver pathologists and hepatologists pursued a consensus on nomenclature for the portal vascular lesions of INCPH. Such standardisation may assist pathologists in the recognition of such lesions, and will possibly facilitate further advancement in this field.

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