# A Case of Non-cirrhotic Portal Hypertension With Antiphospholipid Syndrome

## TL;DR

A 56-year-old man with non-cirrhotic portal hypertension was found to have liver changes linked to antiphospholipid syndrome, and treatment with a shunt and anticoagulation improved his symptoms.

## Contribution

This case report links non-cirrhotic portal hypertension to antiphospholipid syndrome and suggests early TIPS and anticoagulation as potential treatment strategies.

## Key findings

- Liver biopsy confirmed nodular regenerative hyperplasia and obliterative portal venopathy in a patient with non-cirrhotic portal hypertension.
- Antiphospholipid syndrome was identified as a possible underlying cause despite no prior thrombosis history.
- Treatment with TIPS and anticoagulation led to clinical improvement in ascites and breathing difficulties.

## Abstract

Nodular regenerative hyperplasia (NRH) and obliterative portal venopathy (OPV) are two causes of non-cirrhotic portal hypertension (NCPH), which is a vascular liver disease wherein clinical signs of portal hypertension (PHT), such as esophageal varices, ascites, and splenomegaly develop in the absence of cirrhosis and portal vein thrombosis. The etiology often remains unidentified, but herein we present the case of a 56-year-old male with NCPH and refractory ascites who underwent liver biopsy confirming NRH and OPV. Etiological workup revealed beta-2 glycoprotein-1 and anticardiolipin antibodies, concerning antiphospholipid syndrome (APS) despite no prior history of thrombosis. The patient underwent a transjugular intrahepatic portosystemic shunt (TIPS) procedure for his refractory ascites and was started on prophylactic anticoagulation owing to a concern for APS with clinical improvement in his ascites and shortness of breath. Pursuing TIPS earlier in the setting of refractory ascites, as well as offering anticoagulation therapy for patients with possible APS to prevent the development of potential thromboses, could be appropriate recommendations to prevent complications in the disease course. This case report highlights the need for further investigations on the etiologies, diagnosis pathways, and treatment options for NCPH.