Biliarny cirrhosis – the chronic progressing disease which is characterized by gradual replacement of hepatic fabric with fibrous hems. Pathology a long time is not shown in any way, often the diagnosis is made incidentally at screening inspections. At later stages external signs (small hepatic signs), portal hypertensia, defeat of other bodies and systems, hepatic encephalopathy are characteristic. The preliminary diagnosis of cirrhosis and its complications is established on the basis of auxiliary methods of inspection: Ultrasonography, KT of gepatobiliarny system, ERHPG, clinical and biochemical analyses. The final diagnosis is made on the basis of a biopsy of a liver and detection in blood of anti-mitochondrial antibodies. The disease is incurable, at late stages only transplantation of a liver can save the patient's life. Not surgical treatment consists of symptomatic therapy, desintoxication, vitamin therapy and observance of a diet.
Biliarny cirrhosis – the heaviest pathology of a liver at which normal hepatic fabric is replaced with fibrous. This pathology at women after forty years meets more often, and the general prevalence makes 40-50 cases on 1 million population. Close connection of a disease with hereditary factors is noticed – among relatives this pathology meets by 570 times more often. Today the pathogenesis of primary biliarny cirrhosis is up to the end not studied therefore also the exact reasons of its emergence are unknown.
More than in 95% of cases in blood anti-mitochondrial antibodies are defined. However scientists cannot still find out why only mitochondrions of hepatic fabric are surprised, and in other fabrics of it does not occur. Insidiousness of this illness at its hidden beginning – very often biochemical symptoms of a disease can be incidentally revealed at inspection concerning other diseases. The course of a disease progressing at late stages of damage of a liver its change is shown – only in this case possibly to save life of the sick person.
Reasons of biliarny cirrhosis
The modern gastroenterology connects development of primary biliarny cirrhosis with autoimmune violations in an organism. The disease it has the hereditary nature: cases of biliarny cirrhosis are very frequent in the family circle. Tells about the autoimmune nature of a disease also its communication with other diseases of this group: rheumatoid arthritis, tubulyarny renal acidosis, Shegren's syndrome, autoimmune tireoidit and others.
Despite detection of antibodies to mitochondrions practically at all patients with this form of cirrhosis, it was not succeeded to find connection of these antibodies with destruction of cells of a liver. It is known that the disease begins with an aseptic inflammation of bilious ways, the expressed proliferation of an epithelium and the progressing fibrosis in the field of channels. Gradually inflammatory process abates, but fibrosis continues to extend to liver tissue, causing its irreversible destruction.
Provoke the beginning of process and other diseases: congenital malformations and the acquired deformations of bilious ways, cholelithiasis, intra hepatic tumors and the increased lymph nodes in a liver, cysts of a bilious channel, primary skleroziruyushchy or purulent holangit. Also the gramotritsatelny infection – enterobakteriya which have on the surface similar with mitochondrial anti-genes can provoke an onset of the illness. In blood at this disease not only antibodies to mitochondrions and gladkomyshechny cages, but also other immune frustration are defined: increase in level of immunoglobulin M, violation of development In - and T-lymphocytes. All this speaks well for autoimmune pathogenesis of primary biliarny cirrhosis.
Secondary biliarny cirrhosis develops against the background of the diseases resulting in stagnation of bile in extra hepatic bilious ways. Atresias, stones and striktura of bilious channels, cancer of extra hepatic bilious ways, a cyst of a holedokh and concern to them.
Classification of biliarny cirrhosis
The correct classification of cirrhosis is important for development of tactics of further treatment. Function of a liver at biliarny cirrhosis is estimated on a scale of Chayld-Pyyu. In this scale existence of ascites, encephalopathy, figures of bilirubin, albumine and a protrombinovy index (an indicator of the curtailing system of blood) is considered. Depending on score, appropriated for these indicators, distinguish stages of compensation of cirrhosis. The class A is characterized by the minimum quantity of points, life expectancy up to 20 years, survival after band operations more than 90%. In a class In life expectancy it is less, and a lethality after band operations more than 30%. In a class About life expectancy no more than 3 years, and mortality after band operations is more than 80%. Assignment speaks to the patient of a class B or C about need of liver transplantation.
In Russia the scale of METAVIR at which degree of a fibrozirovaniye is estimated on the basis of data of a biopsy, gradation of degree – from 0 to 4 is widely used; The 4th degree speaks about existence of cirrhosis.
Symptoms of biliarny cirrhosis
At early stages the symptomatology of cirrhosis is not specific and erased. So, in most cases complaints begin with a skin itch. More than a half of patients show complaints to a periodic itch of skin, and every tenth is disturbed by an intolerable itch from raschesa on skin. More often the itch develops for half a year, and even several years before emergence of jaundice, but sometimes these two symptoms can arise at the same time. Also characteristic symptoms of the beginning cirrhosis are increased fatigue, drowsiness, dryness of eyes. Occasionally weight and pain in the right podreberye can be the first symptom of a disease. At inspection the increased level of gamma globulins, violation of a ratio of nuclear heating plant to ALT, tendency to arterial hypotension is found.
In process of development of a disease other signs appear. Externally cirrhosis is shown by group of small hepatic signs: vascular asterisks on skin; face reddening, palms and feet; increase in salivary glands; yellowness of skin and all mucous; sgibatelny contracture of hands. Also the patient's nails change – become wide and flat (deformation of nails as "hour glasses"), on them cross whitish strips appear; nail phalanxes are thickened ("drum sticks"). Around joints and on a face, centuries whitish small knots – fatty deposits are formed.
Except external manifestations, symptoms of portal hypertensia appear: due to increase in pressure in a vorotny vein the spleen increases in sizes, in veins of abdominal organs there is a stagnation – the varicosity of gemorroidalny and esophageal veins is formed, on a forward belly wall the expanded venous network as "the head of a jellyfish" is formed. Venous stagnation leads to a liquid propotevaniye in an abdominal cavity with formation of ascites, in hard cases – peritonitis.
The damage of a liver is more expressed, the probability of emergence of complications of cirrhosis is higher. Because of violation of digestion of vitamin D approximately a third of patients has an osteoporosis which is shown pathological changes. Violation of absorption of fats results in deficiency of all fat-soluble vitamins (And, To, D, E) that is shown by symptoms of polyhypovitaminosis. With a stake surplus of fats – is removed. The fifth part of patients has an accompanying hypothyroidism, develop a miyelopatiya and neuropathy. The varicosity of a gullet and rectum can lead to massive bleeding with development of hemorrhagic shock. Damage of a liver leads to dysfunction of other bodies and systems: syndromes, gastro-and a kolopatiya develop gepatorenalny, hepatopulmonary. Because the liver does not perform function of cleaning of an organism of toxins, they freely circulate in blood and make toxic impact on a brain, causing hepatic encephalopathy. Very often in the final of biliarny cirrhosis the gepatotsellyulyarny carcinoma (a malignant tumor), full infertility can develop.
Diagnosis of biliarny cirrhosis
In the presence of signs of stagnation of bile the clinical and biochemical profile is estimated. Increase in level of alkaline phosphatase and G-GTP is characteristic of cirrhosis, the shift of a ratio of ALT and nuclear heating plant, the level of bilirubin increases later. The immune status of the patient is investigated: level of immunoglobulin M, antibodies to mitochondrions increases, increase in level of a rheumatoid factor, antibodies to gladkomyshechny fibers, tissues of a thyroid gland, kernels of cages (anti-nuclear antibodies) is also noted.
Signs of a fibrozirovaniye of a liver can come to light on ultrasonography of a liver and KT of gepatobiliarny system, however the final diagnosis can be made only after carrying out a biopsy of a liver. Even at a research of a bioptat changes in a liver, characteristic of biliarny cirrhosis, can be found only at early stages of a disease, then the morphological picture becomes identical to all types of cirrhosis.
Identification or exception of the diseases leading to development of secondary biliarny cirrhosis is possible by carrying out additional researches: Ultrasonography of abdominal organs, MR-pankreatokholangiografiya, ERHPG.
Treatment of biliarny cirrhosis
The therapist, the gastroenterologist, the hepatologist and the surgeon take active part in treatment of this serious disease. Problems of therapy at cirrhosis is the stop of progressing of cirrhosis and elimination of the phenomena of a liver failure. It is possible to slow down processes of fibrosis by means of immunosupressivny medicines (oppress autoimmune process), bile-expelling means (eliminate ).
Symptomatic therapy consists in the prevention and treatment of complications of cirrhosis: hepatic encephalopathy is eliminated by means of a dietotherapy (reduction of a share of protein, increase in a vegetable component of food), dezintoksikatsionny therapy, reduction of portal hypertensia is reached by means of diuretics. Polyvitaminic medicines are surely appointed, for improvement of digestion enzymes are appointed. At cirrhosis of a class A and B restriction of physical and emotional activities is recommended, and at a class C - a full bed rest.
The most effective method of treatment of biliarny cirrhosis is transplantation of a liver. After change more than 80% of patients survive in the next five years. The recurrence of primary biliarny cirrhosis arises at 15% of patients, however at the most part of patients results of surgical treatment are excellent.
Forecast and prevention of biliarny cirrhosis
It is almost impossible to prevent emergence of this disease, but the refusal of alcohol and smoking, regular inspection at the gastroenterologist in the presence of cases of biliarny cirrhosis in a family, good nutrition and a healthy lifestyle considerably can reduce its probability.
Forecast of a disease adverse. Emergence of the first symptoms of a disease is possible only in ten years from an onset of the illness, however life expectancy at the begun cirrhosis no more than 20 years. Predictively bystry progressing of symptoms and a morphological picture, the advanced age associated with cirrhosis autoimmune diseases is adverse. The most adverse in respect of the forecast is disappearance of an itch, reduction of ksantomatozny plaques, decrease in level of cholesterol in blood.