Sclerosing cholangitis is a disease of the hepatic bile ducts in which chronic inflammation begins in their walls. The result of its occurrence are sclerotherapy, i.e., replacement of scar tissue. This pathology does not have a causal relationship with other liver diseases, but often entails the appearance of complications from this organ. In today's article, we will explain why sclerosing cholangitis develops. Symptoms and treatment of the disease will also be presented to your attention.
Anatomical reference
Bile is an important component of the digestive process. It takes part in the breakdown of fats, increases the activity of pancreatic enzymes and stimulates intestinal motility. Bile is continuously produced by liver cells - hepatocytes. In one day, as a result of their intensive work, about 1 liter of liquid is obtained. After this, bile enters the bladder and duodenum.
The outflow of secretion is organized through special ducts. Depending on the location, they are intrahepatic and extrahepatic. As a result of stagnant processes, the penetration of pathogenic flora, or for a number of other reasons, ducts can become inflamed. At the same time, they talk about the development of a disease such as cholangitis. The pathological process always has a different etiology. Therefore, its following types are distinguished: toxic, bacterial, helminthic, sclerosing. The latter is extremely rare, but is characterized by a severe course.
Sclerosing cholangitis is divided into two forms: primary and secondary. Each of them is characterized by a certain set of signs and course. In the first case, a chronic disease is implied, which is accompanied by stagnation of bile and purulent inflammation of the ducts, their destruction and replacement of connective tissue. The secondary form of pathology develops under the influence of toxic substances. In rare cases, its occurrence is due to insufficient blood supply. In this article, we will dwell in more detail on the primary version of the ailment.
Brief description of the disease
Primary sclerosing cholangitis belongs to the category of rare pathologies of the biliary system. According to statistical information, it is diagnosed in every fourth person per 100 thousand of the population. The mechanism of the development of the disease is reduced to the appearance of an inflammatory process in the small hepatic ducts. In this case, their sclerosis occurs. The ducts are gradually blocked and deformed, through which bile enters the bladder. Stagnant processes extend to the intercellular space of the liver, as a result, cirrhosis develops.
At the end of the last century, it was possible to diagnose the disease only after surgery or an autopsy. Thanks to the development of medicine, today the disease can be detected much earlier. Representatives of the stronger sex at the age of 25 to about 40 years are more exposed to it. These boundaries are very arbitrary, since the disease can be asymptomatic for a long time. Sometimes manifestations of inflammation are mistaken for autoimmune pathologies, ulcerative colitis or cystic fibrosis.
Causes of Cholangitis
The exact causes of the disease are unknown. Doctors distinguish a group of factors that increase the likelihood of its occurrence. These include:
- hereditary predisposition;
- virus activity in the body;
- tendency to autoimmune ailments;
- exposure to toxic substances.
Among the listed factors, genetic mechanisms are given priority. A significant confirmation of this fact are numerous studies of the disease among members of the same family.
Clinical picture
Over the years, the disease can be asymptomatic or with mild symptoms. Patients often cannot tell exactly when the symptoms indicating sclerosing cholangitis appeared . Pathology is usually detected by accident when you see a doctor about other health problems. During diagnosis, the first sign of an ailment is detected - an increase in liver enzymes.
As the disease progresses, the clinical picture also changes. Among its main symptoms, the following should be highlighted:
- weakness, constant drowsiness;
- poor appetite;
- yellowing of the mucous membranes, skin;
- temperature increase to subfebrile values;
- aching pain in the area of the right hypochondrium, extending to the neck or shoulder blade;
- itchy skin;
- multiple xanthomas;
- discomfort in the left hypochondrium due to an enlarged spleen;
- enhanced skin pigmentation.
Sometimes primary sclerosing cholangitis is accompanied by inflammatory bowel pathologies. These include ulcerative colitis, Crohn's disease.
Diagnostic Methods
If you suspect a disease, you should immediately seek help from a doctor. Examination of the patient begins with a study of his medical history, complaints and primary symptoms. After that, they begin a physical examination. The patient may have scratches on the skin, severe jaundice. On palpation, an enlarged liver and spleen are usually found.
In order to confirm the preliminary diagnosis, the patient is sent for further examination. It includes the following activities:
- a blood test (an excess of white blood cell count and ESR indicates an inflammatory process);
- Ultrasound of the abdomen;
- liver elastography (allows you to evaluate the elasticity of the organ);
- retrograde cholangiopancreatography (x-ray using contrast);
- blood biochemistry (with primary sclerosing cholangitis, elevated liver enzymes are observed);
- MRI
- liver biopsy (this research method helps to identify areas of fibrosis).
The listed examination methods confirm the primary sclerosing cholangitis. Diagnosis of this disease also helps determine the severity of the pathological process. There are four of them:
- Gantry. It is characterized by the appearance of fibrosis and swelling of the hepatic ducts.
- Periportal. Symptoms of the first stage are supplemented by more pronounced fibrosis and duct destruction processes.
- Septal. At this stage of the development of the disease, the initial signs of cirrhosis occur.
- Cirrhotic. It is characterized by the full development of biliary cirrhosis.
Based on the results of a comprehensive examination, the doctor prescribes therapy.
Treatment principles
Therapy for this disease is aimed at stopping the inflammatory process, restoring the flow of bile and detoxifying the body. To this end, in modern medicine conservative and surgical methods of treatment are used. In the first case, it means taking medications and following a strict diet. Surgical intervention is indicated in especially serious situations when conservative treatment is ineffective. The choice of a specific method of therapy remains with the doctor.
Drug use
Depending on the severity of the disease, bed rest can be prescribed and any physical activity is excluded. If the patient is worried about severe pain, he is prescribed antispasmodics (No-Shpa, Spazmobryu).
The following drugs help stop the inflammatory process:
- Immunosuppressants ("Azathioprine"). They suppress the activity of the immune system.
- Antifibrogenic agents. Their main action is aimed at eliminating fibrosis and preventing their further development.
- Glucocorticosteroid hormones (Prednisolone). They help reduce inflammatory processes.
The use of these medicines allows you to overcome primary sclerosing cholangitis at the initial stage of development. Symptoms of this disease often interfere with the usual way of life for patients. Itchy skin, gastrointestinal problems and dyspeptic disorders - all these disorders negatively affect their well-being. Therefore, symptomatic therapy is additionally prescribed. It includes hepatoprotectors (Essentiale), gastric enzymes (Creon), and skin pruritus medications. Medicines are always selected individually, taking into account the overall clinical picture and the patient's condition.
Diet features
The patient is assigned food "table number 5". With this diet, you should limit the intake of fatty, fried and spicy foods. It is preferable to replace animal fats with vegetable fats. In addition, you should completely eliminate muffins and sweets, sour fruits and berries, chocolate, alcohol, smoked meats and marinades from the diet.
Allowed to eat lean meat / fish, some types of bread, cereals on the water. You can also eat dairy products, honey, pasta soups on vegetable broth.
With a diagnosis of “primary sclerosing cholangitis,” treatment with medication and diet gives a positive result only at the initial stage. If this time is missed, an operation will be required.
Surgical intervention
Conservative methods of therapy are used for uncomplicated forms of the pathological process. Even a timely visit to a doctor does not always give a positive result in the subsequent therapy. When treatment with drugs does not lead to a normalization of the condition or it is not possible to restore the normal flow of bile, resort to surgical intervention.
Today, doctors prefer endoscopic surgery. They involve all the manipulations through small incisions on the skin. However, such procedures in most cases give a short-term effect and are fraught with complications. They also carry out balloon dilatation with duct stenting. During the procedure, the doctor expands the channels with special cylinders and installs nets that prevent their narrowing. If there is running sclerosing cholangitis, treatment involves a liver transplant.
Possible complications
The disease is characterized by a slow course. It is difficult to treat, and the abundance of systemic manifestations only aggravates the process. Among its most common complications, the following can be distinguished:
- Portal hypertension. This is a pathology accompanied by an increase in pressure in the hepatic bloodstream. Its main manifestation is ascites.
- Cholestasis syndrome. Against the background of sclerosis, the bile ducts gradually narrow, patency is disturbed in them. This explains the appearance of jaundice and skin itching. As the disease progresses, the lumen narrows more and more. Steatorrhea occurs, which is accompanied by osteoporosis.
- Bacterial sclerosing liver cholangitis.
- Chronic pancreatitis.
- Cholangiocarcinoma (a bile duct tumor).
- Cholelithiasis.
Such complications develop at stages 3-4 of the pathological process.
Forecast and preventive measures
Primary sclerosing cholangitis belongs to the category of slowly progressive diseases. Its result in most cases is chronic liver failure. The prognosis of the patient’s advanced age, the presence of concomitant intestinal pathologies, and the occurrence of complications significantly worsen the prognosis. As a rule, from the onset of initial signs to the final stage of the disease, 7 to 12 years pass.
Can primary sclerosing cholangitis be prevented? Reviews of doctors say that due to insufficient study of the disease, specific prevention has not been developed.