Sjogren’s syndrome is an autoimmune systemic lesion of connective tissue characterized by exocrine glandular and extra–glandular manifestations. The most frequent glandular manifestation is a decrease in the secretion of the lacrimal and salivary glands, accompanied by burning in the eyes and dryness of the nasopharynx. Extra-vascular manifestations include myalgia, muscle weakness, arthralgia, hemorrhages, enlarged lymph nodes, neuritis, etc. The diagnosis is made taking into account a complex of clinical and laboratory signs, functional tests. Treatment is carried out with corticosteroid hormones and cytostatics; the course of the disease is more often benign.
Meaning
Sjogren’s syndrome leads among collagenoses in frequency of occurrence and develops significantly more often in women of the age group from 20 to 60 years; in men and in childhood, the disease is less common. The causes of Sjogren’s syndrome are unknown. The most likely determining factors are heredity and an autoimmune response to a viral (presumably rotavirus) infection.
The pathogenetic mechanism of Sjogren’s syndrome consists in the development of an immunoaggressive reaction with the formation of antibodies to their own tissues and lymphoplasmic infiltration of the ducts of the glands of external secretion – salivary, lacrimal, gastrointestinal, etc. In the generalized form of Sjogren’s syndrome, a third of patients develop muscle damage (myositis), kidneys (interstitial abacterial nephritis), blood vessels (productive-destructive, productive vasculitis), lungs (interstitial pneumonia), etc. Sjogren’s syndrome often occurs together with rheumatoid arthritis, Hashimoto’s thyroiditis, systemic lupus erythematosus.
Classification
The course of Sjogren’s syndrome can be subacute and chronic. Taking into account the clinical manifestations and complications, the initial (early), pronounced and late stages of the disease are distinguished. Pathological processes in Sjogren’s syndrome can occur with varying degrees of inflammatory and immunological activity. The high degree of activity of Sjogren’s syndrome is characterized by clinically pronounced phenomena of mumps, keratoconjunctivitis, stomatitis, arthritis; generalized lymphadenopathy, hepatosplenomegaly, laboratory signs of active inflammation.
The moderately active course of Sjogren’s syndrome is characterized by a decrease in inflammation and immunological activity with a simultaneous tendency to destructive changes in the secreting epithelial glands. With minimal activity of the processes, there is a predominance of functional, sclerotic, dystrophic changes in the salivary, lacrimal, gastric glands, which is manifested by severe forms of xerostomia, keratoconjunctivitis, gastritis. In laboratory tests – mild signs of inflammation.
Sjogren’s syndrome symptoms
The appearance of ocular symptoms in Sjogren’s syndrome is due to reduced secretion of tears (tear fluid). At the same time, patients feel burning, “scratching” and “sand” in their eyes. Subjective symptoms are accompanied by itching and redness of the eyelids, accumulation of viscous secretions in the corners of the eyes, narrowing of the eye slits, decreased visual acuity. Dry keratoconjunctivitis develops – inflammation of the cornea together with the conjunctiva of the eye.
Salivary glands in Sjogren’s syndrome increase in size. In a third of patients, as a result of an increase in paired parotid glands, there is a characteristic change in the oval of the face, which has received the name “hamster muzzles” in the literature. Typical symptoms of Sjogren’s syndrome also include dry lips and oral mucosa, stomatitis, congestion, multiple dental caries (more often cervical localization). If in the early stage of Sjogren’s syndrome, dryness of the mucous membranes is noted only during physical exertion and excitement, then in the pronounced period, a feeling of dryness is constantly noted, forcing the patient to often moisten his mouth and drink food.
Examination reveals a bright pink color of the mucous membranes, their slight injury upon contact, dry tongue, a small amount of free saliva of a foamy or viscous nature. Against this background, the addition of a secondary (viral, fungal, bacterial) infection leads to the development of stomatitis. The late stage of Sjogren’s syndrome is characterized by a sharp dryness of the oral cavity, leading to swallowing and speech disorders, cracked lips, keratinization of areas of the oral mucosa, folded tongue, lack of free saliva in the oral cavity.
There is hypofunction of other exocrine glands with the phenomena of dry skin, nasopharynx, vulva and vagina, the development of tracheitis, bronchitis, esophagitis, atrophic gastritis, etc. In Sjogren’s syndrome, there may be a joint syndrome of the type of polyarthralgia or polyarthritis, impaired sensitivity of the feet and hands, neuropathy of the trigeminal nerve and facial nerve, hemorrhagic rashes on the limbs and trunk, fever, myositis, hepatomegaly and splenomegaly.
Diagnostics
From the methods of laboratory diagnostics, a study of the general blood test is carried out, showing moderate leukopenia, anemia, acceleration of ESR. In the biochemistry of the blood in Sjogren’s syndrome, an increase in the level of γ-globulins, total protein, fibrin, seromucoid, sialic acids, the detection of cryoglobulins is determined. Immunological reactions reveal an increase in the level of IgG and IgM immunoglobulins; the presence of antibodies to DNA, LE cells, antibodies to the epithelium of exocrine glands, muscles, collagen, etc.; an increase in the number of B-lymphocytes, a decrease in T-lymphocytes.
In Sjogren’s syndrome, a specific reaction to the Schirmer test is noted – a decrease in tear production is determined in response to stimulation with ammonia. When marking the cornea and conjunctiva with dyes, erosions and dystrophic foci of the epithelium are detected. In Sjogren’s syndrome, contrast radiography (sialography), salivary gland biopsy, ultrasound of the salivary glands, MRI of the lacrimal / salivary glands are performed. To detect complications from other systems, lung x-ray, gastroscopy, ECHO-CG are performed.
Sjogren’s syndrome treatment
The leading role in the therapy of Sjogren’s syndrome is assigned to hormonal (prednisolone) and cytostatic immunosuppressive drugs (cyclophosphamide, chlorbutin) and their combinations (prednisolone + chlorbutin, prednisolone + cyclophosphane). The combination of extracorporeal hemocorrection methods (plasmapheresis, hemosorption, double plasma filtration) with drug therapy in the treatment of Sjogren’s syndrome is indicated in cases of ulcerative necrotic vasculitis, glomerulonephritis, cerebrovasculitis, polyneuritis, and other lesions.
Symptomatic therapy is aimed at eliminating dryness of the mucous membranes and preventing secondary infection. With dry eyes, an artificial tear is prescribed, wearing soft contact lenses, washing the eyes with antiseptic solutions. To normalize salivation, novocaine blockades are carried out, Ca drugs are prescribed; local applications of dimexide, systemic antibiotics and antimycotics are used for parotid gland inflammation.
The affected mucous membranes of the oral cavity in Sjogren’s syndrome need softening and stimulation of regeneration with the help of applications of sea buckthorn and rosehip oils, treatment with ointments (methyluracyl and solcoseryl). In case of secretory insufficiency of the stomach, long-term replacement therapy with hydrochloric acid, natural gastric juice, pepsin is carried out; in case of pancreatic insufficiency, enzyme therapy is prescribed: pancreatin intake, etc.
Prognosis and complications
The course is not life-threatening, but significantly worsens the quality of life. Timely initiated therapy slows down the progression of pathological processes and preserves the ability of patients to work. In the absence of treatment, complications leading to disability are not uncommon.
Primary lesions are often joined by secondary infection with the development of sinusitis, recurrent tracheitis, bronchopneumonia. With systemic lesions, it is possible to develop renal insufficiency, circulatory disorders of the brain and / or spinal cord.
Specific prevention of Sjogren’s syndrome has not been developed.