Autoimmune hepatitis
The content of the article:
- Causes and risk factors
- Forms of the disease
- Symptoms
- Diagnostics
- Treatment
- Possible complications and consequences
- Forecast
Autoimmune hepatitis is a chronic inflammatory immune-dependent progressive liver disease characterized by the presence of specific autoantibodies, increased levels of gamma globulins and a pronounced positive response to immunosuppressive therapy.
For the first time, rapidly progressive hepatitis with an outcome in liver cirrhosis (in young women) was described in 1950 by J. Waldenstrom. The disease was accompanied by jaundice, increased levels of serum gamma globulins, menstrual dysfunction and responded well to adrenocorticotropic hormone therapy. On the basis of antinuclear antibodies (ANA) found in the blood of patients, characteristic of lupus erythematosus (lupus), in 1956 the disease was named "lupoid hepatitis"; the term "autoimmune hepatitis" was introduced almost 10 years later, in 1965.
What the liver looks like with autoimmune hepatitis
Since in the first decade after autoimmune hepatitis was described for the first time, it was more often diagnosed in young women, there is still a misconception that this is a disease of young people. In fact, the average age of patients is 40-45 years, which is due to two peaks of morbidity: at the age of 10 to 30 years and in the period from 50 to 70. It is characteristic that after 50 years, autoimmune hepatitis debuts twice as often as before 30 …
The incidence of the disease is extremely low (nevertheless, it is one of the most studied in the structure of autoimmune pathology) and varies significantly in different countries: among the European population, the prevalence of autoimmune hepatitis is 0.1-1.9 cases per 100,000, and, for example, in Japan - only 0.01-0.08 per 100,000 population per year. The incidence among representatives of different sexes is also very different: the ratio of sick women and men in Europe is 4: 1, in South America - 4.7: 1, in Japan - 10: 1.
Causes and risk factors
The main substrate for the development of progressive inflammatory-necrotic changes in liver tissue is the reaction of immune auto-aggression to its own cells. Several types of antibodies are found in the blood of patients with autoimmune hepatitis, but the most significant for the development of pathological changes are autoantibodies to smooth muscles, or anti-smooth muscle antibodies (SMA), and antinuclear antibodies (ANA).
The action of SMA antibodies is directed against the protein in the smallest structures of smooth muscle cells, antinuclear antibodies work against nuclear DNA and proteins of cell nuclei.
The causal factors for triggering the chain of autoimmune reactions are not reliably known.
Autoimmune hepatitis is caused by the reaction of immune auto-aggression to its own cells
A number of viruses with a hepatotropic effect, some bacteria, active metabolites of toxic and medicinal substances, genetic predisposition are considered as possible provocateurs of the loss of the ability of the immune system to distinguish between "friend and foe":
- hepatitis viruses A, B, C, D;
- Epstein viruses - Barr, measles, HIV (retrovirus);
- Herpes simplex virus (simple);
- interferons;
- salmonella Vi antigen;
- yeast fungi;
- carriage of alleles (structural gene variants) HLA DR B1 * 0301 or HLA DR B1 * 0401;
- taking Methyldopa, Oxyphenisatin, Nitrofurantoin, Minocycline, Diclofenac, Propylthiouracil, Isoniazid and other drugs.
Forms of the disease
There are 3 types of autoimmune hepatitis:
- It occurs in about 80% of cases, more often in women. It is characterized by a classic clinical picture (lupoid hepatitis), the presence of ANA and SMA antibodies, concomitant immune pathology in other organs (autoimmune thyroiditis, ulcerative colitis, diabetes mellitus, etc.), a sluggish course without violent clinical manifestations.
- It has a malignant course, an unfavorable prognosis (by the time the diagnosis is made, cirrhosis of the liver is already detected in 40-70% of patients), it also develops more often in women. The presence in the blood of LKM-1 antibodies to cytochrome P450, LC-1 antibodies is characteristic. Extrahepatic immune manifestations are more pronounced than in type I.
- Clinical manifestations are similar to those of type I hepatitis, the main distinguishing feature is the detection of SLA / LP antibodies to soluble liver antigen.
The existence of type III autoimmune hepatitis is currently being questioned; it is proposed to consider it not as an independent form, but as a special case of type I disease.
The division of autoimmune hepatitis into types does not have significant clinical significance, being more of a scientific interest, since it does not entail changes in terms of diagnostic measures and treatment tactics.
Symptoms
The manifestations of the disease are nonspecific: there is not a single sign that allows one to unequivocally classify it as a symptom of autoimmune hepatitis.
Autoimmune hepatitis begins, as a rule, gradually, with the following general symptoms (sudden onset occurs in 25-30% of cases):
- unsatisfactory general health;
- decreased tolerance to habitual physical activity;
- drowsiness;
- fast fatiguability;
- heaviness and a feeling of fullness in the right hypochondrium;
- transient or permanent icteric staining of the skin and sclera;
- dark coloration of urine (beer color);
- episodes of rise in body temperature;
- decreased or complete lack of appetite;
- muscle and joint pain;
- violations of the menstrual cycle in women (up to the complete cessation of menstruation);
- attacks of spontaneous tachycardia;
- itchy skin;
- redness of the palms;
- pinpoint hemorrhages, spider veins on the skin.
The main symptoms of autoimmune hepatitis are yellowing of the skin and whites of the eyes
Autoimmune hepatitis is a systemic disease in which a number of internal organs are affected. Extrahepatic immune manifestations associated with hepatitis occur in about half of patients and are most often represented by the following diseases and conditions:
- rheumatoid arthritis;
- autoimmune thyroiditis;
- Sjogren's syndrome;
- systemic lupus erythematosus;
- hemolytic anemia;
- autoimmune thrombocytopenia;
- rheumatic vasculitis;
- fibrosing alveolitis;
- Raynaud's syndrome;
- vitiligo;
- alopecia;
- lichen planus;
- bronchial asthma;
- focal scleroderma;
- CREST syndrome;
- overlap syndrome;
- polymyositis;
- insulin-dependent diabetes mellitus.
In about 10% of patients, the disease is asymptomatic and is an accidental finding during examination for another reason, in 30% the severity of liver damage does not correspond to subjective sensations.
Diagnostics
To confirm the diagnosis of autoimmune hepatitis, a comprehensive examination of the patient is carried out.
First of all, it is necessary to confirm the absence of blood transfusions and alcohol abuse in the anamnesis and to exclude other diseases of the liver, gallbladder and bile ducts (hepatobiliary zone), such as:
- viral hepatitis (primarily B and C);
- Wilson's disease;
- lack of alpha-1-antitrypsin;
- hemochromatosis;
- medicinal (toxic) hepatitis;
- primary sclerosing cholangitis;
- primary biliary cirrhosis.
Laboratory diagnostic methods:
- determination of the level of serum gamma globulin or immunoglobulin G (IgG) (increases by at least 1.5 times);
- detection in serum of antinuclear antibodies (ANA), antibodies to smooth muscle (SMA), hepato-renal microsomal antibodies (LKM-1), antibodies to soluble liver antigen (SLA / LP), to asialoglycoprotein receptor (ASGPR), anti-actin (AAAutoantibody)), ANCA, LKM-2, LKM-3, AMA (titer in adults ≥ 1:88, in children ≥ 1:40);
- determination of the level of transaminases ALT and AST blood (increases).
Blood test for autoimmune hepatitis
Instrumental research:
- Ultrasound of the abdominal organs;
- computed and magnetic resonance imaging;
- puncture biopsy with subsequent histological examination of biopsies.
Treatment
The main method of therapy for autoimmune hepatitis is immunosuppressive therapy with glucocorticosteroids or their combination with cytostatics. If the response is positive to the treatment, the drugs can be canceled no earlier than after 1-2 years. It should be noted that after drug withdrawal, 80-90% of patients show reactivation of disease symptoms.
Despite the fact that most patients show positive dynamics during therapy, about 20% remain immune to immunosuppressants. Approximately 10% of patients are forced to interrupt treatment due to complications (erosion and ulceration of the gastric and duodenal mucosa, secondary infectious complications, Itsenko-Cushing's syndrome, obesity, osteoporosis, arterial hypertension, inhibition of hematopoiesis, etc.).
In addition to pharmacotherapy, extracorporeal hemocorrection (volumetric plasmapheresis, cryoapheresis) is performed, which improves the results of treatment: regression of clinical symptoms is observed, the concentration of serum gamma globulins and antibody titers decrease.
Severe cases of autoimmune hepatitis require liver transplant surgery
In the absence of the effect of pharmacotherapy and hemocorrection within 4 years, liver transplantation is indicated.
Possible complications and consequences
Complications of autoimmune hepatitis can include:
- the development of side effects of the therapy, when a change in the "risk - benefit" ratio makes further treatment inappropriate;
- hepatic encephalopathy;
- ascites;
- bleeding from varicose veins of the esophagus;
- cirrhosis of the liver;
- hepatic cell failure.
Forecast
With untreated autoimmune hepatitis, the 5-year survival rate is 50%, and the 10-year survival rate is 10%.
After 3 years of active treatment, laboratory and instrumentally confirmed remission is achieved in 87% of patients. The greatest problem is the reactivation of autoimmune processes, which is observed in 50% of patients within six months and in 70% after 3 years from the end of treatment. After achieving remission without supportive treatment, it can be maintained only in 17% of patients.
With complex treatment, the 20-year survival rate exceeds 80%, with the decompensation of the process, it decreases to 10%.
These data support the need for lifelong therapy. If the patient insists on stopping treatment, dispensary observation is necessary every 3 months.
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Olesya Smolnyakova Therapy, clinical pharmacology and pharmacotherapy About the author
Education: higher, 2004 (GOU VPO "Kursk State Medical University"), specialty "General Medicine", qualification "Doctor". 2008-2012 - Postgraduate student of the Department of Clinical Pharmacology, KSMU, Candidate of Medical Sciences (2013, specialty "Pharmacology, Clinical Pharmacology"). 2014-2015 - professional retraining, specialty "Management in education", FSBEI HPE "KSU".
The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!
