Definition and Causes
Sclerosing cholangitis is an inflammatory disease of the liver, which recognizes its causal element in the inflammation of the bile ducts. The inflammatory process, which can affect the biliary tree at different levels, results in scarring of the intra and / or extrahepatic ducts, in which the bile produced by the liver flows.
Consequently, narrowing (stenosis) and adhesions are formed that prevent the normal biliary outflow in the intestine (from the intrahepatic canaliculi to the common hepatic duct, and from there to the gall bladder and the cystic duct, then to the duodenum). The worsening of the fibrotic processes - rather slow but inexorable - leads, typically over 10-15 years, to the occlusion of the bile ducts with development of hepatic cirrhosis and portal hypertension.
In addition, subjects with sclerosing cholangitis are at greater risk of developing cholangiocarcinoma, a malignant bile duct tumor.
Figure: comparison between the main healthy bile ducts (left) and inflamed (right).
Note the narrowing of the bile ducts caused by the inflammatory process that characterizes the sclerosing cholangitis. From the site: epainassist.com
Sclerosing cholangitis is divided into:
- primary or primitive sclerosing cholangitis : when a disease responsible for biliary fibrosis cannot be identified → often the damage is considered immunomediated, as evidenced by the frequent association of primary sclerosing cholangitis and diseases with autoimmune etiology, eg. ulcerative colitis, Crohn's disease, chronic autoimmune pancreatitis, sarcoidosis. Other pathogenetic theories, today less credited, attribute a rolo to bacterial toxins and to the same infectious agents. Primary sclerosing cholangitis is about twice as common in men than women and is rare in children, manifesting itself electively from the third to the sixth decade of life.
- secondary sclerosing cholangitis : when it results from a clinically identifiable underlying disease → among the main causes of secondary sclerosing cholangitis, the presence of calculi inside the bile ducts, recurrent pancreatitis, surgery on the biliary tree, AIDS or injection of chemotherapy drugs through the hepatic artery.
Symptoms
To learn more: Symptoms Sclerosing Cholangitis
Some patients may be asymptomatic for many years, and continuous monitoring may be necessary in these cases. The clinical picture associated with sclerosing cholangitis is extremely variable, but generally progressive and complicated - in most cases - from the evolution towards a picture of liver cirrhosis with severe impairment of liver function.
Jaundice, itching, weight loss, weakness, poor appetite and hepatic tenderness (pain in the right hypochondrium), are the classic symptoms of onset, which over time can be complicated by evolving into those typical of portal hypertension and liver failure: edema, ascites, esophageal varices, hemorrhoids, splenomegaly.
Diagnosis and treatment
In the face of clinical suspicion, sclerosing cholangitis is diagnosed through an examination known as perendoscopic retrograde cholangio-pancreatography (ERCP), during which a contrast medium is injected directly into the biliary tree so that the structure can be better studied through radiographic images . A flexible tube equipped with a video camera and light source is sent down through the mouth until it reaches the duodenum, in particular up to the papilla of Vater (where the biliary and pancreatic ducts enter the intestine). Through this sphincter structure the contrast medium necessary to study the structure of the biliary tract through X-ray images is injected. For the monitoring of patients with sclerosing cholangitis already diagnostic it is possible to use the cholangio-pancreatography with magnetic resonance (MRCP), which produces images of the biliary tree exploiting the high intensity of the bile signal (which appears white), compared to the surrounding tissues that generate little signal (therefore they appear dark). CT can also play a complementary role to cholangiography.
Since the fibrotic process of the bile ducts is not reversible, drug therapy is based on the control of associated symptoms. The itching, for example, can be controlled by the use of antihistamines and prevented by the use of bile acid sequestering resins.
- Corticosteroids, aziatoprine, penicillamine and methotrexate have given variable results and are associated with important toxic effects. Ursodeoxycholic acid (ursodiolo) can reduce itching and improve biochemical parameters, but it has not been shown to alter the natural history of the disease
The integration of the diet with fat-soluble vitamins (vitamin A, D, E and K) into tablets prevents specific deficiencies, deriving from their reduced absorption due to the lack of bile emulsifying activity. Antibiotics can be taken to prevent episodes of bacterial cholangitis (gall bladder infection), very common in people with sclerosing cholangitis.
Liver transplantation remains the only definitive treatment to resolve a primitive sclerosing cholangitis, reserved for the most serious cases complicated by liver failure. The minimally invasive laparoscopic and endoscopic surgery (operative ERCP) can still help: the doctor can place stents in correspondence of the biliary obstructions, dilating the walls, or provide for the removal of the same to then weld the residual ends of the bile duct .
