Overview
Hepatoportal sclerosis (HPS) is a rare liver condition also known as idiopathic non-cirrhotic portal hypertension (INCPH), obliterative portal venopathy, or non-cirrhotic portal fibrosis. In this disease, the small blood vessels inside the liver — especially the tiny branches of the portal vein — become scarred and narrowed over time. This raises the blood pressure in the portal vein, the large vessel that carries blood from the gut to the liver. Importantly, the liver itself does not become cirrhotic (severely scarred) the way it does in common liver diseases like alcoholic liver disease or hepatitis. Because blood pressure builds up in the portal system, the body tries to reroute blood through other vessels. This leads to enlarged veins in the esophagus (called varices), an enlarged spleen, and a buildup of fluid in the abdomen (ascites). People may also develop low blood counts because the enlarged spleen traps blood cells. Fatigue, abdominal discomfort, and easy bruising are common early complaints. Treatment focuses on managing the complications of high portal blood pressure rather than curing the underlying problem. Medications called beta-blockers help reduce the risk of bleeding from varices. Procedures like endoscopic banding can treat varices directly. In severe cases, a procedure called TIPS (transjugular intrahepatic portosystemic shunt) or even liver transplantation may be considered. With careful monitoring and treatment, many people with HPS can live for many years.
Also known as:
Key symptoms:
Swollen or enlarged spleen (splenomegaly)Enlarged veins in the esophagus or stomach (varices) that can bleedVomiting blood or passing black, tarry stools due to variceal bleedingBuildup of fluid in the abdomen (ascites)Fatigue and low energyEasy bruising or bleedingLow red blood cell count (anemia)Low white blood cell and platelet countsAbdominal discomfort or fullnessYellowing of the skin or eyes (jaundice) — less common than in cirrhosisSwelling in the legs (edema)
Clinical phenotype terms (30)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Hepatoportal sclerosis.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Hepatoportal sclerosis at this time.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Hepatoportal sclerosis.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What is causing my hepatoportal sclerosis — is there an underlying condition or medication that triggered it?,Do I have varices, and what is the best plan to prevent them from bleeding?,How often do I need endoscopy and imaging to monitor my condition?,Should I be on a beta-blocker, and what side effects should I watch for?,What dietary changes do I need to make, especially regarding salt and alcohol?,At what point would you consider a TIPS procedure or liver transplant evaluation?,Are there any clinical trials or new treatments I should know about?
Common questions about Hepatoportal sclerosis
What is Hepatoportal sclerosis?
Hepatoportal sclerosis (HPS) is a rare liver condition also known as idiopathic non-cirrhotic portal hypertension (INCPH), obliterative portal venopathy, or non-cirrhotic portal fibrosis. In this disease, the small blood vessels inside the liver — especially the tiny branches of the portal vein — become scarred and narrowed over time. This raises the blood pressure in the portal vein, the large vessel that carries blood from the gut to the liver. Importantly, the liver itself does not become cirrhotic (severely scarred) the way it does in common liver diseases like alcoholic liver disease or h
How is Hepatoportal sclerosis inherited?
Hepatoportal sclerosis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Hepatoportal sclerosis typically begin?
Typical onset of Hepatoportal sclerosis is adult. Age of onset can vary across affected individuals.
Which specialists treat Hepatoportal sclerosis?
1 specialists and care centers treating Hepatoportal sclerosis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.