ncpf

Non cirrhotic portal fibrosisSHANKAR ZANWAR

Intrahepatic causes of portal hypertension

Definition

Banti (1898) – synd. of splenomegaly and hypersplenism PHT and anaemia in the absence of haematological disease.

Non cirrhotic portal fibrosis/ idiopathic portal hypertension is Disease of unknown etiology Involves of small and medium branches of portal vein presence of portal hypertension Liver structure and function primarily remains normal

NCPF recommendations APASL, Hep Intl, 2007

Epidemiology


Etio-pathogenesis factors

Unknown, ultimately cause portal venous injury

Environmental – Poverty Arsenic exposure – in 10% of cases Low levels of ADAMTS13 levels

A Goel, C Eapen, IJG, 2016

Agents - Infections – endotoxins

Host Prothrombotic diorders Autoimmunity – ↓ T1 lymphocytes, CD8 lymphocytes

Sarin S K, J Hep 2014

Possible etiological factors

Infections – bacterial, protozoal, schistosomiasis

Drugs – Azathioprine, Vinyl chloride, copper sulphate(nila thotha), didanosine, vitamin A

Prothrombotic states – MPD(JAK 2), MTHFR etc

Immune diseases – SLE, scleroderma, celiac disease

Pathogenesis animal model

R Khanna, SK Sarin, J of Hep, 2014

Hemodynamics


Age and gender


Clinical manifestations

Mass in LUQ – 12-69% Splenomegaly – 26-97% Anemia – 90% Variceal bleed – 32-84% - commonest Palpable liver – 33-54% Decompensation – 8- 20% Ascites – 9- 34% Edema feet – 4-18%


Lab findings

Hypersplenism – 27-87% Abnormal LFT – 18-73% High bilirubin – 9- 31% Deranged INR – 4-81% Hypoalbuminemia – 16% Autonomic dysfunction – 25 – 67% ↓α adrenergic

response


Radiological features No e/o cirrhosis

Subcapsular parenchymal atrophy

Hyperechoic bands surrounding portal vein branches

Peripheral pruning of PV br, sudden cut-off of 2nd and 3rd degree branches – withered tree appearance

Splenomegaly, gamma gandy bodies

Gurkaynak, j clin USG, 1998

Histopathology Phleboscleosis in 2/3 of PV – 40-100%

Dilated portal venules – 40-70%

Porto-portal fibrosis – 30-60%

Portoseptal fibrosis – 17-60%

NRH/FNH – 20-70%

Dilated sinusoids - 90%

Histological hallmark – Obliterative portal venopathy


Diagnostic criteria

APASL criteria Presence of mod – sev splenomegaly E/o of PHT – varices of collaterals Doppler – patent – spleno-portal axis and HV LFTs – normal Biopsy - no e/o cirrhosis/ parenchymal injury

Other features Absence of signs of CLD Absence of viral markers

HIV and NCPF Prevalence NCPF/IPH in HIV around 0.45 – 1%

May be due to HAART especially didanosine Recurrent opportunistic infections hypercoagulability HIV per se due to infection of stellate cells

Predominantly in males (50-100%), MSMs or prolonged infection (mean >11.5y) and IRIS

HIV-NCPF patients are older, ↓ plts and CD4, ↑ LFTs

Median kPa – 9.5, median HVPG 8mmHg

LTx need reported

Cachay, Br J Med, 2011

Natural history

Long term survival after bleed management – 80-100%

In 25-33%, slow parenchymal atrophy decompensation

Decompensations/complications – over 7 yrs after diagnosis

PVT 46% Ascites 50% Liver failure 21%

Hillaire, Gut 2002

Portal biliopathy – 9-40% of cases

Management Acute episodes

Volume resuscitation

Vasoactive drugs – somatostatin, octreotide and terlipressin

EVL and EST effective 80-90% Glue for GOV-2 and IGV1

Secondary prophylaxis – NSBB and EVL equal efficacy NSBB – 47% ↓ in grade of varices, 18 % minor S/e

Sarin SK, Gastroenterology, 2010

Prevention of bleed

Primary prophylaxis All patients with moderate to massive splenomegaly,

with suspected NCPF should have screeing OGD

Primary EVL recommended for large varices but no consensus on NSBB

BRTO is an option for large gastric varices

Decompressive surgery is not recommended for primary prophylaxis

Secondary prophylaxis

TIPS – uncontrollable bleed, recurrent variceal hemorrhages despite endotherapy and severe PHG bleed

Surgical shunt – When accompanied by EHPVO – shunt surgeries

effective mode of treatment Prevents recurrent bleeds, improves

hypersplenism, with minimal risks of HE

Differences between NCPF and IPH

NCPF IPHGender Equal F>M 3:1Age (Mean) 30y 40-45Autoimmune factors

Rare Common

Parenchymal nodules

Common Rarer

Bile duct proliferation

Common Rarer

WHVP Normal HigherHematemesis 94% 40%


NCPF mimickers

Hepatic schistosomiasis Causatives – S. Mansoni, S. Japonicum

Larval form reside in superior rectal tributaries, later in SMV

Liver disease occurs due to entrapment of eggs in portal venules (<50mm) granulomatous inflammation fibrosis – Symmers pipe stem fibrosis

4-8% of these develop PHT

Altered Th1/Th2 response

C/f of schistosomiasis are simliar to NCPF or EHPVO

Diagnosis – eggs in stools or rectal Bx

Stool micorscopy – Kato Katx method

Serological tests using ELISA of egg antigens

USG – thickened PV, septae along PV giving fish scale network appearnace

CT shows capsular and septal calcification , irregular hepatic countour

Natural history correlates with no. of eggs that parallels with eggs in stool

Treatment

Praziquantel – complete resolution in 28.5% over 3-5 years - ↓ spleen size, SMV and SV diameter

Recent metanalysis – artemesinin superior to praziquantel

PHT – treatment as i NCPF

Devascularisation surgeries better hemodynamic improvement than DSRS in schistosomiasis.

Congenital hepatic fibrosis Rare developmental disorder

Abnormal ductal plate formation, irregular bile ducts periportal fibrosis

Majority (64%) a/w ARPKD, 25.6% a/w Caroli’s synd. remaining are isolated

Median age at ∆ - 0-20 years

Those a/w Caroli’s present with cholangitis(86%) and PHT(34%)

Esopahgeal varices and hyperseplnism is common

Ascites, HE and HPS is rare

Increased risjk of cholangioca.

No relation between ARPKD severity and liver involvement

LFTs usually normal except for cholangitis

Imaging – dilated IHBR, enlarged left lobe, splenomagly +/- cysts in liver and kidney

PHT treatment as in NCPF

Definitive treatment is SLKT

Nodular regenerative hyperplasia

Cause of NCPH in Europe(27%) and Japan(14%)

Higher (7X) in people > 80 years

Cause Chemotherpay Immunosuppresants Inflammator disorders Neoplastic disorders

Pathogenesis – adaptive hyperplastic reaction of hepatocytes to functional/mechanical alteration in portal flow

HPE – complete/partial transformation of parenchyma into regenerative nodules with characteristic absence of septae

PHT – result of presinusoidal compression

Majority asymptomatic, symptomatics – c/f of PHT with preserved liver function

Imaging nonspecific – nodules, variable enhancement , MRI – hyper in T1, hypo/iso on T2

Rx – treatment of cause, PHT – endotherapy/ TIPS.

Thank you

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