Mikulich’s disease (Sjögren’s disease) is a rather rare chronic disease that manifests itself as a parallel increase in all salivary and lacrimal glands and their further hypertrophy.
Pathology Description
The main factors underlying its development are viral infection, blood diseases, allergic and autoimmune processes, and malfunctions of the lymphatic system. This disease occurs only in adults, mainly in women. It was first described by the German surgeon I. Mikulic in 1892. Now it is believed that the enlargement of the glands is not an independent disease, but a concomitant syndrome with various kinds of disorders of the endocrine system.
Causes of the disease
Scientists still have not figured out the exact reasons underlying the Mikulich disease. Only hypothetical reasons are advanced, for example:
Autoimmune disease;
· The first stage of development of a malignant tumor;
Disruption of the hematopoietic system;
Tuberculosis
Syphilis;
Mumps (mumps);
Epidemic encephalitis.
The widespread damage to organs and systems of the body violates the neurovegetative regulation of the lacrimal and salivary glands, and changes their secretory function. Autoimmune or allergic reactions contribute to clogging of the excretory ducts of the glands with eosinophilic plugs, delay the secret, and reduce the ducts of smooth muscle and myoepithelial cells. As a result, interstitial and lymphoid tissues grow, squeeze the ducts and lead to an ever-increasing hypertrophy of the salivary and lacrimal glands. Let's look at the symptoms of Mikulich's disease.
Symptoms of the disease
Most often, the disease appears in the age period from 20 to 30 years. Elderly people are less likely to suffer; in children, it has not been recorded. In the first stages, the symptoms are similar to signs of mumps, in addition, if inflammatory complications occur, it can be triggered.
The first and most important symptom of Mikulich’s disease is swelling of the lacrimal glands. Gradually, they become painful when pressed, and in some cases so increase in size that under their weight the eyeball drops and even bulges forward. Although the consistency of the glands is quite dense, suppuration is not observed.
The second symptom is an increase in the salivary glands (submandibular, parotid, less often sublingual). Usually this process is two-sided, swelling occurs on both sides, and only in exceptional cases - on one. Often there is an increase in lymph nodes.
The third symptom is complaints of dry mouth, dry conjunctivitis, and multiple dental caries. In the case of a typical course of the disease, the liver and spleen increase, leuko- and lymphocytosis are observed.
Diagnosis of the disease
Mikulich’s disease is diagnosed by doctors from the general clinical picture. Often a sialogram is made, revealing dystrophic changes in glandular tissue, well showing an increase in salivary glands, narrowing of their excretory ducts. If they were not injured, it is necessary to carefully examine the lymphomas of the orbits.
Widespread use also puncture histobiopsy. Histologically, it is possible to detect hyperplasia of the lacrimal and salivary glands, to determine the atrophic modification of the parenchyma and the presence of lymphoid infiltration of the stroma.
Parallel studies of blood around the lymph nodes and analysis of bone marrow puncture are highly effective for diagnosing and developing treatment regimens.
It is worth noting that during Mikulich’s disease (the treatment of which we will consider below), the capsule of the gland is not affected, therefore, the tissues of the salivary and lacrimal glands do not connect to the mucous membranes and skin, thanks to this factor, this syndrome can be distinguished from various kinds of productive chronic inflammations.
Laboratory blood tests show a picture characterizing lymphoproliferative diseases, and the results of a urinalysis usually do not reveal any pathologies.
Using computed tomography, it is possible to more accurately determine the structure and size of the salivary glands, to exclude the appearance of malignant neoplasms.
Diagnosis of the disease includes an immunochemical and immunological examination with an examination by an allergist-immunologist, as well as consultation with an ophthalmologist, performing a Schirmer test and taking samples with fluorescein.
Treatment
Treatment of Mikulich's disease should be under the supervision of a hematologist. The main remedy is arsenic preparations, most often a solution of sodium arsenate in a 1% concentration. It is used for subcutaneous injections, starting from 0.2 milliliters and gradually increasing the dosage to 1 milliliter once a day. By the end of treatment, the dose is reduced. For complete therapy, approximately 20-30 injections are required. In the same doses, the drug "Duplex" is used. Two to three times a day, the patient is given potassium arsenate for oral administration. The course of treatment lasts about three to four weeks. You can also drink arsenic tablets, use dopan and myelosan.
Additional methods
Compresses on the affected glands and antibiotics are widely used. In addition to drug therapy, blood transfusions are also used. In some cases, the achievement of positive dynamics becomes possible due to x-ray therapy, which stops the inflammatory process and temporarily reduces the size of the glands, restores their secretory function, and eliminates dry mouth. The overall strengthening of the body contributes to the intake of vitamins.
We examined the features of the disease and Mikulich syndrome.