HealthDiseases and Conditions

Primary sclerosing cholangitis: symptoms, diagnosis and treatment

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 sclerosing, that is, replacement by scar tissue. This pathology has no causal relationship with other liver diseases, but often entails complications on the part of this organ. In today's article we will tell you why cholangitis develops sclerosing. Symptoms and treatment of the disease will also be presented to your attention.

Anatomic Help

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 peristalsis. Bile continuously produces liver cells - hepatocytes. For one day as a result of their intensive work, about 1 liter of liquid is obtained. After this, the bile enters the bladder and duodenum.

The outflow of the secret is organized by means of 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 causes, ducts can become inflamed. Thus they speak about the development of such a disease as cholangitis. The pathological process always has a different etiology. Therefore, the following types are distinguished: toxic, bacterial, helminthic, sclerosing. The latter is extremely rare, but 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 characteristics and current. In the first case, a chronic disease is implied, which is accompanied by stagnation of bile and non-ductal inflammation of the ducts, their destruction and replacement by 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 disease.

Brief description of the disease

Primary sclerosing cholangitis belongs to the category of rare pathologies of the bile excretory system. According to statistical data, 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. Gradually, the ducts through which bile enters the bladder overlap and deform. Stagnant processes extend to the intercellular space of the liver, as a result of which cirrhosis develops.

At the end of the last century, the disease was diagnosed only after surgery or autopsy. Thanks to the development of medicine today, the ailment can be discovered much earlier. To a greater extent, he is exposed to members of the stronger sex at the age of 25 to about 40 years. These boundaries are very conventional, since the disease can last for a long time asymptomatically. Sometimes manifestations of inflammation are mistaken for autoimmune pathologies, 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. Among them are:

  • Hereditary predisposition;
  • Activity of viruses in the body;
  • Propensity to autoimmune ailments;
  • Exposure to toxic substances.

Among the listed 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 ailment can be asymptomatic or with poorly expressed symptoms. Patients often can not tell exactly when exactly the sclerosing cholangitis symptoms appeared. Pathology is usually identified by chance when you see a doctor about other health problems. During the diagnosis, the first sign of a malaise is found - an increase in the parameters of liver enzymes.

As the disease progresses, the clinical picture changes. Among its main symptoms are the following:

  • Weakness, persistent drowsiness;
  • Poor appetite;
  • Yellowing of mucous membranes, skin;
  • Increase in temperature to subfebrile values;
  • Aching pain in the zone of the right hypochondrium, giving to the neck or scapula;
  • Itching;
  • Multiple xanthomas;
  • Discomfort in the left hypochondrium, arising from the increase in the spleen;
  • Strengthened skin pigmentation.

Sometimes primary sclerosing cholangitis accompanies inflammatory bowel pathology. These include ulcerative colitis, Crohn's disease.

Methods of diagnosis

If you suspect a disease, you should immediately seek help from a doctor. The examination of the patient begins with a study of his history, complaints and primary symptoms. Then they start physical examination. The patient can have scratching on the skin, severe jaundice. When palpation is usually found enlarged liver, spleen.

To confirm the preliminary diagnosis, the patient is sent for further examination. It includes the following activities:

  • Blood test (excess of white blood cells and ESR indicative of inflammatory process);
  • Ultrasound of the abdominal cavity;
  • Elastography of the liver (allows to assess the elasticity of the organ);
  • Retrograde cholangiopancreatography (X-ray examination using contrast);
  • Biochemistry of blood (with primary sclerosing cholangitis, there are overestimated liver enzymes);
  • MRI;
  • Liver biopsy (this method of investigation helps to identify areas of fibrosis).

The above survey methods allow us to confirm the primary sclerosing cholangitis. Diagnosis of this disease also helps to determine the severity of the pathological process. There are four in total:

  1. The portal. It is characterized by the appearance of fibrosis and swelling of the hepatic ducts.
  2. Periportal. Symptoms of the first stage are supplemented with more pronounced fibrosis and processes of destruction of the ducts.
  3. Septal. At this stage of the disease, there are initial signs of cirrhosis.
  4. Cirrhotic. Characterized by the full development of biliary cirrhosis.

Based on the results of the complex examination, the doctor prescribes therapy.

Principles of treatment

Therapy in this disease is aimed at arresting the inflammatory process, restoring the bile flow and detoxifying the body. To this end, conservative and surgical methods of treatment are used in modern medicine. In the first case, it means taking medications and adhering to 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.

Application of medicines

Depending on the severity of the disease, bed rest can be prescribed and any physical exertion is excluded. If the patient is concerned about severe pain, he is prescribed spasmolytic drugs (No-shpa, Spasmobryu).

The following preparations help to stop the inflammatory process:

  1. Immunosuppressants ("Azathioprine"). They suppress the activity of the immune system.
  2. Antifungal agents. Their main action is aimed at eliminating fibrosis and preventing their further development.
  3. Glucocorticosteroid hormones (Prednisolone). They contribute to reducing inflammatory processes.

The use of these medicines allows to overcome at the initial stage of development the primary sclerosing cholangitis. Symptoms of this disease often interfere with the usual way of life for patients. Skin itching, gastrointestinal problems and dyspeptic disorders - all these disorders negatively affect their health. Therefore, in addition, symptomatic therapy is prescribed. It includes the use of hepatoprotectors (Essentiale), gastric enzymes (Creon), and preparations for eliminating skin itching. Medicines are always selected individually, taking into account the overall clinical picture and the condition of the patient.

Diet Features

The patient is assigned food "table number 5". With this diet should limit the consumption of fatty, fried and spicy dishes. Animal fats are preferable to replace with vegetable. In addition, you need to completely exclude from the diet muffins and sweets, sour fruits and berries, chocolate, alcohol, smoked meat and marinades.

Allowed to eat lean meat / fish, some types of bread, porridge on the water. You can also eat dairy products, honey, pasta soups on vegetable broth.

With the 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 in uncomplicated forms of the pathological process. Even a timely call 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 current of bile, resort to surgical intervention.

Today, doctors prefer endoscopic surgery. They assume the carrying out of 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 performed. During the procedure, the doctor widens the channels with special balloons and sets the meshes that prevent their narrowing. If there is a triggered sclerosing cholangitis, the treatment involves a liver transplant.

Possible complications

The disease is characterized by a slow flow. It does not respond well to therapy, and the abundance of systemic manifestations only adds to the process. Among the most common complications of it are the following:

  1. Portal hypertension. This pathology, accompanied by increased pressure in the hepatic blood flow. Its main manifestation is ascites.
  2. Cholestasis syndrome. Against the backdrop of sclerosis, the bile ducts gradually narrow, and their permeability is impaired. This explains the occurrence of jaundice and skin itching. As the disease progresses, the lumen tapers more and more. There is steatorrhea, which is accompanied by osteoporosis.
  3. Bacterial sclerosing cholangitis of the liver.
  4. Chronic pancreatitis.
  5. Cholangiocarcinoma (bile duct tumor).
  6. Cholelithiasis.

Such complications develop on 3-4 stages of the pathological process.

Prognosis and prevention measures

Primary sclerosing cholangitis belongs to the category of slowly progressing diseases. Its result in most cases is chronic liver failure. Significantly worsen the prognosis of the patient's advanced age, the presence of concomitant abnormalities of the intestine, the occurrence of complications. As a rule, from the moment of appearance of initial signs to the final stage of the disease passes from 7 to 12 years.

Is it possible to prevent primary sclerosing cholangitis? The doctors' comments indicate that due to insufficient study of the disease, no specific prevention has been developed.

Similar articles

 

 

 

 

Trending Now

 

 

 

 

Newest

Copyright © 2018 en.birmiss.com. Theme powered by WordPress.