Sclerosing cholangitis is a disease of the hepatic bile ducts, in which chronic inflammation begins in their walls. The result of its occurrence are the processes of sclerosis, i.e., replacement by scar tissue. This pathology has no causal relationship with other liver diseases, but often leads to complications from this organ. In today's article we will tell you why sclerosing cholangitis develops. Symptoms and treatment of the disease will also be presented to your attention.
Anatomical reference
Bile is an important part 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. The bile then enters the bladder and duodenum.
The outflow of the secret is organized through special channels. Depending on the location, they areintrahepatic and extrahepatic. As a result of stagnant processes, the penetration of pathogenic flora, or for a number of other reasons, the ducts can become inflamed. At the same time, they talk about the development of such a disease as cholangitis. The pathological process always has a different etiology. Therefore, the following types of it 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 non-purulent inflammation of the ducts, their destruction and replacement with 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 variant of the disease.
Brief description of the disease
Primary sclerosing cholangitis belongs to the category of rare pathologies of the biliary system. According to statistics, 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 through which bile enters the bladder gradually overlap and deform. Congestive processes spread to the intercellular space of the liver, resulting in cirrhosis.
Even at the end of the last century, it was possible to diagnose the disease only after surgery or autopsy. Thanks to the development of medicine today, the disease can be detected much earlier. To a greater extent, it affects the stronger sex aged 25 to about 40 years. These boundaries are very arbitrary, since the disease can be asymptomatic for a long time. Sometimes manifestations of inflammation are mistaken for autoimmune diseases, ulcerative colitis or cystic fibrosis.
Causes of cholangitis
The exact causes of the development of the disease are unknown. Doctors identify a group of factors that increase the likelihood of its occurrence. These include:
- hereditary predisposition;
- viral activity in the body;
- prone to autoimmune diseases;
- exposure to toxic substances.
Among these factors, genetic mechanisms are of primary importance. A weighty confirmation of this fact are numerous studies of the disease among members of the same family.
Clinical picture
For many years, the disease can be asymptomatic or with mild signs. Patients often cannot say exactly when the symptoms indicating sclerosing cholangitis appeared. Pathology is usually detected incidentally when visiting a doctor for other he alth problems. During the diagnosis, the first sign of the disease is detected - an increase in liver enzymes.
According to the measureAs the disease progresses, the clinical picture also changes. Among its main symptoms, the following should be highlighted:
- weakness, constant sleepiness;
- poor appetite;
- yellowing of mucous membranes, skin;
- increase in temperature to subfebrile values;
- aching pain in the area of the right hypochondrium, radiating to the neck or shoulder blade;
- skin itching;
- multiple xanthomas;
- discomfort in the left hypochondrium due to an enlarged spleen;
- increased 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 anamnesis, complaints and primary symptoms. After that, proceed to the physical examination. The patient may have scratching on the skin, severe jaundice. Palpation usually reveals an enlarged liver and spleen.
In order to confirm the preliminary diagnosis, the patient is sent for further examination. It includes the following activities:
- blood test (an excess of leukocytes and ESR indicates an inflammatory process);
- Ultrasound of the abdominal cavity;
- liver elastography (allows you to assess the elasticity of the organ);
- retrograde cholangiopancreatography (X-ray examinationusing contrast);
- blood biochemistry (with primary sclerosing cholangitis, elevated levels of liver enzymes are observed);
- MRI;
- liver biopsy (this research method helps to identify areas of fibrosis).
The listed examination methods allow to confirm primary sclerosing cholangitis. Diagnosis of this disease also helps to determine the severity of the pathological process. There are four in total:
- Portal. It is characterized by the appearance of fibrosis and swelling of the hepatic ducts.
- Periportal. Symptoms of the first stage are complemented by more pronounced fibrosis and destruction of the ducts.
- Septal. At this stage in the development of the disease, the initial signs of cirrhosis appear.
- Cirrotic. It is characterized by the full development of biliary cirrhosis of the liver.
Based on the results of a comprehensive examination, the doctor prescribes therapy.
Principles of treatment
Therapy for this disease is aimed at stopping the inflammatory process, restoring bile flow and detoxifying the body. For this purpose, conservative and surgical methods of treatment are used in modern medicine. 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.
Applicationmedicines
Depending on the severity of the disease, bed rest may be prescribed and any physical activity excluded. If the patient is worried about severe pain, he is prescribed antispasmodics ("No-shpa", "Spasmobrew").
The following drugs help to 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 inflammation.
The use of the listed medications allows you to overcome primary sclerosing cholangitis at the initial stage of development. The symptoms of this disease often interfere with the normal way of life for patients. Skin itching, problems with the gastrointestinal tract and dyspeptic disorders - all these disorders negatively affect their well-being. Therefore, symptomatic therapy is additionally prescribed. It includes taking hepatoprotectors (Essentiale), gastric enzymes (Creon) and drugs to eliminate itching. Medicines are always selected individually, taking into account the overall clinical picture and the patient's condition.
Features of the diet
The patient is assigned food "table number 5". With this diet, you should limit the consumption of fatty, fried and spicy foods. It is preferable to replace animal fats with vegetable fats. In addition, it is necessary to completely eliminatea diet of muffins and sweets, sour fruits and berries, chocolate, alcohol, smoked meats and marinades.
Low-fat meat / fish, some types of bread, porridge on the water are allowed. You can also eat dairy products, honey, pasta soups in vegetable broth.
When diagnosed with primary sclerosing cholangitis, treatment with medication and diet gives a positive result only at the initial stage. If this time is missed, surgery will be required.
Surgery
Conservative methods of therapy are used for uncomplicated forms of the pathological process. Even a timely visit to the doctor does not always give a positive result in the subsequent therapy. When treatment with drugs does not lead to normalization of the condition or it is not possible to restore the normal flow of bile, they resort to surgical intervention.
Today, doctors prefer endoscopic operations. They involve carrying out all manipulations through small incisions on the skin. However, such procedures in most cases give a short-term effect and are fraught with complications. Balloon dilatation with stenting of the ducts is also carried out. During the procedure, the doctor expands the channels with special balloons and installs meshes that prevent their narrowing. If advanced sclerosing cholangitis is present, treatment involves a liver transplant.
Possible Complications
Diseasecharacterized by slow flow. It is difficult to treat, and the abundance of systemic manifestations only aggravates the process. Among its most common complications are the following:
- Portal hypertension. This is a pathology accompanied by an increase in pressure in the hepatic circulation. Ascites is considered its main manifestation.
- Cholestasis syndrome. Against the background of sclerosis, the bile ducts gradually narrow, and their patency is impaired. 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 cholangitis of the liver.
- Chronic pancreatitis.
- Cholangiocarcinoma (tumor of the bile ducts).
- Cholelithiasis.
Such complications develop at 3-4 stages of the pathological process.
Forecast and preventive measures
Primary sclerosing cholangitis is classified as a slowly progressive disease. Its result in most cases is chronic liver failure. The prognosis is significantly worsened by the advanced age of the patient, the presence of concomitant intestinal pathologies, and the occurrence of complications. As a rule, from the moment the initial symptoms appear to the final stage of the disease, it takes from 7 to 12 years.
Can primary sclerosing cholangitis be prevented? Doctors' comments indicate that due to insufficient study of the disease, specific prevention has not been developed.
