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

Abstract

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.

Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

“…The imaging examinations showed hepatic atrophy, splenomegaly and ascites in both diseases. The common clinical manifestations included abnormal liver-function tests, hypersplenism and varicosis 14 . Some patients presented anaemia and thrombocytopenia due to hypersplenism.…”

Performance of spleen stiffness measurement by 2D-shear wave elastography in evaluating the presence of high-risk varices: comparative analysis of idiopathic portal hypertension versus hepatitis B virus

“…On the one hand, SS provided the highest AUC compared with LS and SS/LS; on the other hand, although no significant difference was observed between the AUCs of SS, LSPS, PH risk score and varices risk score, the SS by using 2D-SWE could be more easily performed in clinical compared with other parameters. When patients are in the early stage of HBV-infections, the hallmarks of liver are mostly present in the hepatocytes, as opposed to the portal tracts in IPH [ 17 , 18 ]. Additionally, the volume of hepatocytes accounts for more than 90% of the total volume of the liver, with liver function being more severely affected in HBV-infected patients than in those with IPH.…”

Attach importance to the individualized treatment of adult portal hypertension based on etiology and pathogenesis: A review

“…INCPH is a rare disease characterized by PH without cirrhosis, liver disease of other causes, or visceral vein thrombosis. [ 36 ] In general, the potential pathogenesis of INCPH can be divided into 5 categories: immune-based diseases, chronic infection, exposure to drugs or toxins, genetic diseases, and a hypercoagulable state. [ 37 ] Diagnosis of INCPH relies on exclusion diagnosis, including: clinical findings of symptoms or signs related to PH; eliminating the possibility of chronic hepatitis and liver cirrhosis; exclusion of portal vein thrombosis, Budd-Chiari syndrome, congenital liver fibrosis, and other causes of noncirrhotic PH; and histological manifestations of INCPH.…”