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.
Description of pathology
The main factors underlying its development are a viral infection, blood diseases, allergic and autoimmune processes, disorders in the lymphatic system. This disease occurs only in adults, mainly in women. It was first described by the German surgeon I. Mikulich in 1892. It is now believed that glandular enlargement is not an independent disease, but a concomitant syndrome with various kinds of endocrine system disorders.
Causes of disease
Scientists still have not figured out the exact causes underlying Mikulich's disease. Only hypothetical grounds are put forward, for example:
autoimmune disease;
First stage of malignant tumor development;
Disorders of the hematopoietic system;
TB;
syphilis;
mumps (mumps);
epidemicencephalitis.
The widespread damage to the organs and systems of the body disrupts the neurovegetative regulation of the lacrimal and salivary glands, changes their secretory function. Autoimmune or allergic reactions contribute to the clogging of the excretory ducts of the glands with eosinophilic plugs, retain the secret, and reduce the ducts of smooth muscle and myoepithelial cells. As a result, the interstitial and lymphoid tissues proliferate, compress the ducts, and lead to ever-increasing hypertrophy of the salivary and lacrimal glands. Let's look at the symptoms of Mikulich's disease.
Disease symptoms
Most often the disease appears in the age period from 20 to 30 years. Older people suffer less often, it has not been recorded in children. In the first stages, the symptoms are similar to those of chronic parotitis, in addition, if inflammatory complications occur, it can be provoked.
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 they increase in size so much that under their weight the eyeball drops and even protrudes 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 bilateral, swelling occurs on both sides, and only in exceptional cases - on one side. Often there is an increase in lymph nodes.
Third symptom- 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, leukocytosis and lymphocytosis are observed.
Diagnosis of disease
Mikulich's disease is diagnosed by doctors from the general clinical picture. Often a sialogram is made, which reveals dystrophic changes in the glandular tissue, which clearly shows an increase in the salivary glands, a narrowing of their excretory ducts. If they are not affected, it is necessary to carefully examine the lymphomas of the orbits.
Puncture histobiopsy is also widely used. Histologically, it is possible to detect hyperplasia of the lacrimal and salivary glands, to determine atrophic modifications of the parenchyma and the presence of lymphoid infiltration of the stroma.
Highly effective for diagnosis and development of a treatment regimen are parallel studies of blood around the lymph nodes and analysis of bone marrow puncture.
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 with the mucous membranes and skin, thanks to this factor, this syndrome can be distinguished from various kinds of productive chronic inflammation.
Laboratory blood tests show a picture that characterizes lymphoproliferative diseases, and urine tests usually do not reveal any pathologies.
With the help of computed tomography, you can more accurately determine the structure and size of salivaryglands, exclude the appearance of malignant neoplasms.
Diagnosis of the disease includes 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 at 0.2 milliliters and gradually increasing the dosage up 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, 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. Taking vitamins contributes to the overall strengthening of the body.
We examined the features of the disease and Mikulich's syndrome.
