We recently encountered a case of rheumatoid arthritis combined with non-sclerotic portal hypertension in the ward, and we review the knowledge of non-sclerotic portal hypertension as follows.
Idiopathic portal hypertension (IPH) is also known as non-cirrhotic portal fibrosis, non-sclerotic portal hypertension or hepatic portal sclerosis. It is a disease of portal hypertension caused by occlusive fibrosis and sclerosis of the intrahepatic portal vein branches, which often manifests clinically as splenomegaly, anemia and upper gastrointestinal bleeding, but without cirrhosis and extrahepatic portal vein obstruction. The etiology is still unknown. It has been reported that patients mostly have a background of poor environmental hygiene or recurrent intestinal infections from early childhood, and it is also believed that long-term exposure to or ingestion of arsenic, ethylene chloride or cytotoxic drugs (azathioprine, leucovorin, etc.) is related, and it is proposed that bacterial antigens and toxic substances can cause intrahepatic portal vein branch root or sinusoidal endothelial damage, producing venous inflammation or thrombosis playing an important role in the pathogenesis. The disease is more common in India and Japan, and accounts for about 3% to 4% in Western countries.
Clinical manifestations
Patients are mostly young men and middle-aged women. The onset of the disease is insidious, and the time of onset is often unclear.
Splenomegaly: All patients have splenomegaly of varying degrees, often accompanied by hypersplenism.
2. Upper gastrointestinal bleeding: due to portal hypertensive varices, more than 2/3 of them were detected by endoscopy as esophageal varices.
3.Anemia: Most of them have significant anemia and its related symptoms.
4. Liver: palpable but no significant enlargement, jaundice and ascites were rare. There are no signs of chronic liver disease such as siderosis, gynecomastia and skin pigmentation.
5. Laboratory tests: anemia is orthocytic or hypocytic, leukopenia and thrombocytopenia are also common, and liver function tests are normal or mildly abnormal.
Diagnostic points]
The main diagnostic basis of this disease are.
1. There are clinical signs of splenomegaly, anemia and upper gastrointestinal bleeding.
2. There is more than one kind of hematocrit reduction.
3.Liver function test is normal or nearly normal.
4, Endoscopy or barium swallow on X-ray confirms the presence of varicose veins.
5.Real-time or color Doppler ultrasound confirms dilated portal vein trunk and open collateral circulation, splenomegaly with increased blood flow in splenic vein, but the liver is homogeneous in texture and there is no sign of cirrhosis.
6.Special examination
(1) Direct or indirect portal venography: It is the best method to assess the circulation of the portal system. It shows
(1) Decrease in the number of medium-sized portal vein branches in the liver, narrowing or abrupt truncation of the roots of small portal vein branches, and sparse or irregular peripheral or subperitoneal fine branches;
(ii) Enlargement of the main trunk of the portal vein without obstruction;
(③) The lateral branches are open, and the left gastric vein is often seen to flow away from the liver.
(2)Measurement of portal vein pressure (PVP) and embedded hepatic vein pressure (WHVP): PVP is significantly elevated and exceeds 20 cmH2O, WHVP is mildly elevated and significantly lower than PVP, suggesting that the disease is an intrahepatic pre-sinusoidal obstruction.
(3) Isotope scintigraphy: 99mTc-SC liver and spleen radiography showed hepatosplenomegaly, but not bone marrow, which is the main difference between this disease and cirrhosis.
(4) Laparoscopy and liver tissue biopsy: laparoscopy showed a slightly non-conforming liver envelope, but no diffuse nodules. Liver tissue microscopy shows fibrosis and sclerosis of medium-sized portal branches, organic thrombosis and recanalization of some portal branches, fibrosis around the confluent area, and sometimes fibrous bundles are seen extending into the liver parenchyma in the form of needles, but the lobular structure remains normal and there are no diffuse regenerative liver nodules.
It is not necessary to have each of the above to diagnose the disease, but to confirm the diagnosis, all etiologies of cirrhosis, schistosomiasis liver fibrosis and extrahepatic portal vein obstruction must be absolutely excluded.
Treatment and prognosis
The main treatment is for portal hypertension, and the effect is better than that of cirrhotic decompensation.
1.Drugs to reduce portal pressure such as Propranolol (Takeaway), spironolactone, isosorbide mononitrate (ISMN) or isosorbide nitrate (cardiac pain). Choose the combination of two drugs on weekdays, refer to the chapter of portal hypertension.
2.Therapeutic treatment with Chinese medicine such as activating blood circulation and removing blood stasis, benefiting Qi and nourishing Yin.
3.Endoscopic sclerosing agent injection or ligation of varicose veins.
4.Partial splenic artery embolization.
5.Surgical treatment can be done by portal chi dissection or portal body shunt.
The disease usually does not progress to cirrhosis, most can live a normal life, 50% of patients can survive up to 25 years since the beginning of the disease. The prognosis depends on the effectiveness of the prevention and treatment of variceal bleeding.