Schmidt's syndrome is an autoimmune disease that occurs in 30 people per 1 million. At the same time, 75% of patients are women. This is a rather complex and severe pathology with multiple lesions of the endocrine system. For the first time this disease was described back in 1926 by Schmidt A., and even then the researcher was able to describe almost all concomitant diseases of the autoimmune polygladular syndrome of the second type.
general description
Pathology most often occurs against the background of adrenal insufficiency, that is, they produce an insufficient amount of hormones: aldosterone and cortisol. It is these hormones that are responsible for metabolic processes in the human body.
Symptom complex can occur against the background of insufficient functioning of the sex glands. This pathology is also called hypogenitalism, that is, the body has a very low synthesis of sex hormones.
The work of the parathyroid gland can also be suppressed, that is, the production of hormones that are responsible for the amount of calcium in the blood is practically inactive. This type of pathology is called hypoparathyroidism.
It is also possible to reduce the amount of thyroxine and triiodothyronine associated with the inflammatory process that occurs in the thyroid gland. Such a disease is called lymphocytic thyroiditis.
Schmidt syndrome manifests itself in each person can be different. Some patients have completely no symptoms, and some have severe disorders. Very often, type 1 diabetes joins the syndrome.
Classification
To date, there are three types of syndrome, and each of them has certain symptoms.
A type | Symptomatology | Risk group |
1 | Addison's Disease hypoparathyroidism, chronic candidiasis of the skin | This type most often already manifests itself in childhood. Skin problems are observed at the age of 5 years. Hypoparathyroidism is formed by 10 years. And by 15, there are already problems with the adrenal glands |
2 | This type is also characterized by unstable work of the adrenal glands, type 1 diabetes, hypothyroidism or hyperthyroidism may appear | At risk are persons over 30 years old, with women |
3 | Most often characterized by the presence of a history of hypothyroidism, persistent gastrointestinal disorders can torment. In some patients, stable weight loss is observed, in others - a stable weight gain. | May appear at any age |
Reasons for the development of pathology
Even during the first studies of Schmidt's syndrome, it was noticed that pathology does not always appear sporadically. Very often, the disease was detected immediately in several family members, which was the reason to assume that the pathology has a genetic nature of origin. Further observations confirmed this theory, and it was found that pathology is formed against the background of a defect in the structure of the gene responsible for the encoding of the autoimmune protein regulator. This happens in the 21st pair of chromosomes, this leads to the fact that the body does not have an immune response to its own antigens.
Another factor in the development of the disease is a lack of suppressor T-lymphocytes in the body. Which also leads to the inability of the body to resist its own antibodies.
Symptomatology
One of the main symptoms that accompanies Schmidt syndrome is insufficient adrenal function. Against this background, the patient may feel severe fatigue, weakness in the muscles, loss of appetite. As a rule, there is a reduced resistance to infections. A person can rapidly lose weight. The skin becomes dry, hyperpigmentation may occur, especially in areas of the body that are open. Pigmentation discoloration may occur on the mucous membranes.
Symptoms of Schmidt syndrome are also:
- tachycardia;
- frequent urination, especially at night;
- loss of sex drive;
- hypotension;
- hyperhidrosis;
- asthenia;
- diarrhea;
- feeling of nausea up to vomiting.
Also, the patient can observe himself, which has become less attentive.
Accompanying illnesses
In fact, the symptoms in the endocrinology of Schmidt syndrome are so diverse that it is difficult to describe them. Related diseases include:
- red blood cell aplasia;
- malignant myasthenia gravis (up to 30%);
- lack of immunoglobulin A in the body;
- vitiligo (found in almost 25% of patients);
- diabetes, occurs in 40-50% of patients;
- hypogenitalism, that is, underdevelopment of the genitals;
- sprue.
With the development of lymphocytic thyroiditis, the following symptoms can be observed:
- thyroid enlargement;
- pain in the front of the neck, pressure;
- drowsiness, swelling and pallor of the skin against the background of a decrease in hormone production.
If a diffuse toxic goiter is formed against the background of the syndrome, then the patient can observe the following symptom complex:
- disturbances in the work of the heart muscle;
- poor tolerance to hot weather;
- weakness and headaches;
- severe swelling;
- proliferation in the size of the thyroid gland;
- change of nails;
- tremor.
Due to Schmidt's syndrome, celiac disease, pleurisy, pituitary neoplasms, pericarditis can occur.
Diagnostic measures
At the first stage of diagnosing the syndrome, the doctor collects an anamnesis, clarifies the symptoms and finds out if the relatives have such a disease.
Physical examination gives a complete picture of dermatological disorders, problems with nails, weight changes and other external manifestations of the disease.
A blood test is taken to determine the basic hormones in the body, the presence of antibodies and to determine the amount of glucose.
Instrumental methods include: ultrasound of the pelvic organs and thyroid gland. Computed tomography is performed to determine the condition of the adrenal glands. In some cases, depending on the symptoms, consultation with specialized specialists may be required.
Therapeutic measures
You should be aware that the treatment of Schmidt syndrome is only symptomatic, it is completely impossible to get rid of this pathology. Most often, compensatory therapy for adrenal insufficiency is required. Treatment in this case consists of taking glucocorticoids and mineralocorticoids.
Subsequently, synthesized hormones, in particular, “L-thyroxine,” are used in therapy. The dosage of this drug is always selected individually, depending on the dynamics of the condition. In the presence of diabetes, insulin is prescribed.
In adjuvant therapy, vitamin D and ascorbic acid, calcium are used.
Forecast
As mentioned earlier, the disease is incurable, so the patient will have to spend all his life on hormone replacement therapy, in which "L-thyroxine" is most often used. The further prognosis completely depends on the degree of damage to the internal organs and associated diseases.
Lack of treatment can cause death. If the patient complies with all the recommendations of the attending doctor, then there is a great chance to live to a very old age. Although in some cases, replacement therapy does not give the expected results and disability occurs.
Patients with this type of pathology will have to be regularly observed by an endocrinologist. Surveys are recommended twice a year. If a difficult moment comes in life associated with emotional overload, then you will have to increase the dose of hormones for this period.
Due to the genetic nature of the origin of the disease, no prophylaxis exists for this syndrome.