Autoimmune pancreatitis: causes, diagnosis and treatment
This type of pancreatitis affects not only the pancreas, but also other organs. Most often affected by lymph nodes, bile ducts, lungs, salivary glands, kidneys.
Autoimmune pancreatitis develops due to an active pathological process, resulting in it being autoimmune antibodies or killer cells that are aggressive against healthy body tissues.
The process of damage and destruction of normal tissues is an autoimmune inflammation, which is caused by deterioration of the immune defense. Most often remains with a person for life, turning into a chronic form with periods of exacerbation and remission. Autoimmune pancreatitis is precisely this form and refers. During inflammation, the exocrine function of the organ decreases, pseudocysts and calcification in the gland tissues are formed. Sclerosis often causes the development of diabetes.
Autoimmune pancreatitis occurs in both sexes, but is twice as common in men as in women. Patients vary greatly in age, most of them older than 50 years.
Symptoms
It is interesting that in the autoimmune form of pancreatitis there are no sharply defined signs and a sharp deterioration in the state of well-being during exacerbation. Sometimes the disease is asymptomatic and the diagnosis is made only when complications occur.
The following symptoms stand out:
- Discomfort in the abdomen and pain sensations that have a girdling character. Duration varies: from several minutes to several hours. The pain is either intense or of a moderate nature, which occurs as a result of taking fatty, spicy food or alcohol. Jaundice of mucous membranes, skin and biological fluids released by the body. .The development of jaundice is associated with impaired bile flow into the duodenum in the presence of narrowed pancreatic ducts:
- The color of the stool becomes lighter.
- Urine, on the contrary, darkens.
- Saliva is colored yellow.
- Appears skin itching.
- Appetite decreases. There is nausea, vomiting.
- Increased gas generation.
- Sensation of bitterness and dryness in the mouth.
- Appearance of unpleasant odor from the mouth.
- The body weight decreases.
- Asthenic syndrome manifests itself: decreased efficiency, increased fatigue, weakness, depressed mood, decreased appetite.
- Light. In them, the tissue becomes denser, which leads to the appearance of dyspnea. The patient has a rapid breathing.
- Kidney. Renal failure develops, protein appears in the urine, which should not be normal.
- Liver. The tissue is tightened, but without tumor cells. With palpation, this is easily detected. The patient has complaints of severity in the right upper quadrant, regardless of whether he took food or not.
- Salivary glands become inflamed, the tissues are scarred. There is dryness in the mouth, soreness of the glands. It becomes difficult to talk, eat, swallow.
Forms of the disease
Traditionally, the "gold standard" for the diagnosis of autoimmune pancreatitis is histology. Based on this study, a microscope is allocated:
- Pancreatitis is lymphoplasmocytic sclerosing. This form is more common in older men. Yellowness of the mucous membranes and skin appears, the pancreas is affected. It is well treated with steroid preparations.
- Pancreatitis is idiopathic, protocol-concentric with granulocyte epithelial damage. It appears in younger men and women.
These two forms can only be distinguished from each other when conducting a study under a microscope.
Based on concomitant autoimmune diseases that develop with damage to other organs:
- Pancreatitis is an autoimmune isolated. Only the pancreas is affected.
- Syndrome of autoimmune pancreatitis. In addition to the pancreas, there are other organs with autoimmune disease. Based on the location of the lesion, the following is defined:
- Diffusive form in which almost the entire pancreas is affected.
- Focal form. More often, the lesion is localized in the head of the pancreas.
Reasons for
The cause of the disease is unknown. In consequence of a malfunction in the body, the immune system begins to attack its own organs.
Autoimmune pancreatitis is often associated with rheumatoid arthritis, Sjogren's syndrome, and inflammatory bowel disease.
Diagnosis
For the correct diagnosis the patient should be examined by a team of experienced specialists in autoimmune pancreatitis, including a gastroenterologist, endoscopist, therapist and endocrinologist.
Autoimmune pancreatitis occurs twice as much in men as in women. Its appearance usually occurs at the age of 50-60 years, but in some patients it can develop as early as 30 years.
The disease can occur either alone or in conjunction with other autoimmune disorders, including sclerosing cholangitis, primary biliary cirrhosis, inflammatory bowel disease, rheumatoid arthritis, hypothyroidism, etc. In addition, autoimmune pancreatitis has been observed concomitantly with retroperitoneal fibrosis. The general symptoms of autoimmune pancreatitis include jaundice, weight loss, and mild abdominal pain. Severe abdominal pain or other symptoms of acute pancreatitis are uncommon. Nevertheless, autoimmune pancreatitis occurs with periods of exacerbation, alternating with remission.
To make a diagnosis, an anamnesis of the patient's illness and life is collected. Note the difference in lost body weight. Conduct palpation of the pancreas and adjacent organs.
Determination of immunoglobulin concentration IgG4
When examining blood, attention is drawn to such an indicator as IgG4 immunoglobulin. When everything is normal in the body, then its level does not exceed 5% of the total blood serum. As soon as its level rises, it can be safely asserted about the presence of diseases in which the infiltration of organs by plasma cells that produce IgG4 occurs.
That is, there is tissue inflammation - fibrosis and scarring. One form of systemic disease is autoimmune pancreatitis.
High concentration of IgG4 is observed in almost 90% of patients with autoimmune pancreatitis, and its increase can be tens of times.
Laboratory tests :
- The total blood test is aimed at studying the sedimentation rate of erythrocytes and blood protein.
- Blood glucose level. If there is a violation of the secretory function of the pancreas, the glucose value is higher.
- Lipidogram. The amount of fat-like substances is estimated in the blood. Biochemical analysis of blood.
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- Oncotest on markers of a malignant tumor.
- General analysis of urine. Determine the presence of bile pigments. The results of the analysis are necessary for assessing the state of the genitourinary system.
- Blood test for the concentration of immunoglobulin G4, a protein that rises in autoimmune reactions.
- Stool analysis is done to identify undigested food residues and fat content. Refine the percentage of elastase and chymotrypsin( pancreatic enzymes that break down proteins).
Methods of investigation
- ultrasound. It allows to make accurate measurements of the organ, to evaluate the structure of the pancreas, spleen and liver. Identifies the causes of violations of the outflow of bile, the presence of stones and tumors.
- Spiral computed tomography. The study is based on the performance of several images taken in a row at different depths. Very clear images of the organs are obtained. Autoimmune pancreatitis presents itself with increased diffuse areas and a narrowed excretory duct.
- MRI.The method is similar in many respects to the previous study( SCT).
- X-ray examination of the bile excretory system using contrast medium. More often this method is used if there are difficulties with the diagnosis or its specification.
- Biopsy. You can not fully consider it reliable, for the reason that in the pancreas during inflammation normal areas alternate with damaged or altered ones. Perhaps a piece of tissue will be taken from healthy tissue and this can affect the formulation of the final diagnosis. In autoimmune pancreatitis, the patient contains a large number of plasmocytes that contain IgG4.
Basic diagnostic criteria for :
- Visual inspection( palpation and ultrasound examination).
- Serology( blood test to determine the level of IgG4).
- Histological examination( biopsy of the pancreas).
Therapy for autoimmune pancreatitis
Inflammation of the pancreas can be treated conservatively, in this case, no surgical intervention is required. Because of the strong similarity of symptoms, autoimmune pancreatitis is often taken for malignant formation, hence it leads to unnecessary pancreatic resection.
Approximately 1/3 of patients underwent surgery for suspected pancreatic cancer, but eventually found that they were benign tumors.
In this subgroup of patients, glucocorticosteroid therapy prevents the development of the disease and within a few weeks restores the normal functioning of the pancreas.
When pancreatitis of any form is strictly recommended compliance with the diet.
In order to ensure that the body does not aggressively compete with its own organs, immunosuppressants are prescribed.
To remove pain, spasms, discomfort are called - antispasmodics.
Helping to cope with the digestion of food and establish a normal chair helps the reception of enzyme preparations.
Treatment with the use of drugs with ursodeoxycholic acid is aimed at improving the outflow of bile and for the gradual recovery of liver cells.
If the gastric mucosa is damaged, the doctor prescribes drugs that reduce the production of hydrochloric acid. They resort to the help of proton pump inhibitors.
In the development of diabetes - insulin.
Surgery may be required if the pancreatic ducts are too narrow, which prevents a normal outflow of pancreatic juice. Treatment with glucocorticosteroids in this case will not help. Do stenting the ducts through the stent, so the operation is fairly easy, with a quick recovery of the patient.
Evaluation of the quality of therapy, includes improvement in the indicators of clinical signs and symptoms:
- Reducing jaundice, bloating and back pain.
- Pancreas decreased in size on the image.
- The level of pancreatic enzymes and total bilirubin decreased.
- The normal level of glucose in the blood and the level of insulin has been restored.
Complications and consequences
Autoimmune pancreatitis often remains with unexplained pathogenesis. The increase in serum IgG4 and the diagnostic criteria for autoimmune pancreatitis do not reflect the full spectrum of the disease. The steroid disease is responsive, but the maintenance of remission continues to be difficult.
Complications of autoimmune pancreatitis :
- The violation of the mechanism of absorption of nutrients in the intestine, leads to: protein deficiency, hypovitaminosis, exhaustion of the body.
- Disorder of water-salt metabolism( thirst, occurrence of swelling in the lower extremities, dryness of mucous membranes and skin).
- Subhepatic jaundice when there is a violation of the outflow of bile.
- Infections. Because of inflammation, changes in the density of pancreatic tissue, the appearance of toxins in the blood, pathogens, it is possible to develop: sepsis, peritonitis, purulent cholangitis, inflammatory infiltrates.
- Erosive and ulcerative formations in the digestive tract.
- Elevated pressure in the portal vein due to poor blood flow from the liver, contributes to the development of chronic obstruction of the duodenum.
- The effusion and accumulation of fluid in the peritoneal cavity causes pancreatogenic ascites.
- There is a high probability of developing a cancerous tumor.
What are the consequences of the disease? Practically always it is possible to reduce them to a minimum. In the conduct of adequate and timely treatment, it is possible in many cases to achieve complete restoration of the pancreas and to establish its work.
Even if long-term treatment was required, the organ underwent significant attack and damage, then with therapy it is possible to stop the progression and development of the process.
Forecasts for this form of pancreatitis should be based on the severity and number of complications. It is necessary to consider a lot of factors, such as: the defeat of organs in consequence of the attack of the immune system, whether diabetes developed.
The prevention of autoimmune pancreatitis is difficult to talk about, because the causes and factors affecting its development are not established.
The outcome of the treatment and diagnosis of any disease is highly dependent on the patient and the doctor who leads the patient. The patient is obliged to comply with all the prescriptions of the specialist, and he, in turn, to apply the most sparing and effective treatment.
Useful video about pancreatitis and nutrition in this disease
