Related articles: Sclerosing cholangitis
Definition
Sclerosing cholangitis is a disease that affects the bile ducts, ie the channels responsible for transporting the bile produced by the liver towards the gall bladder and the intestine.
This pathological condition derives, in particular, from a chronic inflammatory reaction which, over time, causes a decrease in the caliber (stenosis) and a scarring (fibrosis) of the biliary tract. This results in the presence of intra- and extra-hepatic ducts with an irregular course, with points of obstruction alternated with stretches of dilation, which hinder the passage of bile necessary for the digestion of fats. The stagnation of the latter predisposes to damage to the liver tissue, which manifests itself in the form of cirrhosis, portal hypertension and, sometimes, cholangiocarcinoma (bile duct cancer).
Sclerosing cholangitis can be primary (without known cause) or secondary to immune dysfunctions (congenital or acquired), infections (eg cytomegalovirus), connective tissue diseases and abuse of some drugs (eg floxuridine). Other factors that can predispose to the development of this condition are the congenital anomalies of the biliary tree, the formation of stones (choledocholithiasis), ischemic damage and postoperative stenosis (for example, following liver transplantation), cancer processes and parasitic infestations.
Sclerosing cholangitis is frequently associated with inflammatory bowel disease (especially ulcerative rectocolitis, rarely Crohn's disease).
Most common symptoms and signs *
- Anorexia
- Ascites
- Asthenia
- Cachexia
- Bad digestion
- Colaluria
- Diarrhea
- Yellow Diarrhea
- Abdominal pain
- Pain in the upper part of the abdomen
- Edema
- Hepatitis
- Hepatomegaly
- Temperature
- I made clear
- Swollen legs
- Abdominal swelling
- Portal hypertension
- hypovitaminosis
- Jaundice
- Nausea
- Osteoporosis
- itch
- Water retention
- Drowsiness
- splenomegaly
- steatorrhea
- Triad of Charcot
Further indications
Sclerosing cholangitis presents a chronic and slowly progressive course.
Onset is generally insidious and the affected subject may remain asymptomatic for several years, before manifesting the characteristic signs of cholestasis (stagnation of bile within the hepatic biliary system), such as jaundice and widespread itching. During the course of the disease, alterations may occur due to the deficiency of fat-soluble vitamins, steatorrhea, feeling of exhaustion and general malaise.
Cholestasis can be complicated by the overlapping of infectious processes, which lead to the appearance of fever and abdominal pain in the upper right quadrant.
Persistent cutaneous and / or scleral jaundice indicates an advanced state of the disease.
The terminal phase of sclerosing cholangitis involves cirrhosis, portal hypertension, ascites and liver function failure. Furthermore, patients with long-standing conditions have a greater risk of developing bile duct tumors (cholangiocarcinoma) than the general population.
The finding of altered blood tests, possibly associated with clinical symptoms, is not sufficient to define the diagnosis of sclerosing cholangitis. The evaluation requires instrumental investigations to visualize the characteristic deformity of biliary tract features, such as ultrasonography, endoscopic retrograde cholangiopancreatography (ERCP) and magnetic resonance imaging. Usually, these tests are associated with a biopsy that shows intrahepatic damage.
Treatment focuses on removing blockages in the ducts and improving bile flow by endoscopic or surgical techniques.
In more advanced cases, liver transplantation may become necessary, which improves life expectancy in patients suffering from sclerosing cholangitis.
