Sjogren ‘s disease is a systemic autoimmune disease related to connective tissue diseases; characterized by the defeat of many secreting glands, mainly salivary and lacrimal.
Also isolated syndrome , Sjogren’s (loss of lacrimal and salivary glands) accompanying rheumatoid arthritis, diffuse diseases of connective tissue, biliary system diseases and other autoimmune diseases.
Sjogren ‘s disease is the most common disease among connective tissue diseases and occurs in women 10-25 times more often than in men, usually at the age of 20-60 years, much less common in children.
The causes of the disease are unknown. Most researchers consider Sjogren’s disease as a consequence of immunopathological reactions to a viral infection, presumably retroviral .
Symptoms of Sjogren’s disease can be divided into glandular and extra-iron .
Glandular manifestations are due to the defeat of secreting glands, characterized mainly by a decrease in their function.
A permanent sign of damage to the lacrimal glands in Sjogren’s disease is eye damage associated with a decrease in the secretion of the tear fluid. Patients complain of burning sensations, “scratches” and “sand” in the eyes. The itching of the eyelids, redness, and congestion in the corners of a viscous white discharge are often noted. Later, photophobia, narrowing of the palpebral fissures appear, visual acuity decreases. An increase in the lacrimal glands in Sjogren’s disease is rarely observed.
The second mandatory and permanent symptom of Sjogren’s disease is the defeat of the salivary glands with the development of chronic inflammation. It is characterized by dry mouth and an increase in salivary glands.
Often, even before the appearance of these signs, the red border of the lips, stickiness , stomatitis , enlargement of the nearby lymph nodes, and multiple (usually the cervical) caries of the teeth are noted . In one third of the patients, a gradual increase in the parotid glands is observed, leading to a characteristic change in the oval of the face, described in the literature as “hamster’s muzzle” or “chipmunk’s muzzle”.
In the initial stage of the disease, dry mouth appears only during physical exertion and agitation.
In the pronounced stage, dry mouth becomes constant, accompanied by the need to drink dry food, the desire to moisten the mouth during a conversation. The mucous membrane of the mouth becomes bright pink, easily injured. There is little free saliva, it is frothy or viscous. Tongue dry. The lips are covered with crusts, there are signs of inflammation, a secondary infection can occur, including fungal and viral infections. Characterized by multiple cervical caries of teeth.
The late stage is manifested by a sharp dry mouth, the inability to speak, to swallow food without washing it down with liquid. The lips of such patients are dry, in cracks, the mucous membrane of the oral cavity with symptoms of keratinization, folded tongue, free saliva in the oral cavity is not detected.
Dry nasopharynx with the formation of dry crusts in the nose, in the lumen of the auditory tubes can lead to temporary deafness and the development of otitis . Dryness of the pharynx, as well as vocal cords causes hoarseness.
Frequent complications are secondary infections: sinusitis , recurrent tracheobronchitis and pneumonia . The lesion of the glands of the external genital organs is observed in about 1/3 of patients with Sjogren ‘s disease . The mucous membrane of the vagina is reddened, dry, and often patients suffer from burning pains and itching.
A frequent sign of Sjogren’s disease is dry skin. Sweating can be reduced.
Violation of swallowing due to the presence of dry mucous membranes. Many patients develop chronic atrophic gastritis with severe secretory insufficiency, clinically manifested by severity and discomfort in the epigastric region after eating, belching air, nausea, decreased appetite. Less common pain in the epigastric region.
There is a direct relationship between the degree of dryness and inhibition of gastric secretory function. The defeat of the biliary tract ( cholecystitis ) and liver ( hepatitis ) is observed in most patients. There are complaints of heaviness and pain in the right hypochondrium, bitter taste in the mouth, nausea, and poor tolerance to fatty foods.
Involvement in the pancreas process (pancreatitis) is manifested by pain and indigestion.
Vlezhelezistye manifestations of Sjogren’s disease are very diverse, are systemic. Pain in the joints, slight stiffness in the morning. Signs of muscle inflammation (muscle pain, moderate muscle weakness, a slight increase in the level of creatine phosphokinase in the blood) are observed in 5-10% of patients.
In most patients with Sjogren ‘s disease , there is an increase in the submandibular, cervical, occipital, supraclavicular lymph nodes, and in 1/3 of the patients the increase in lymph nodes is common. In the latter case, an increase in the liver is often detected.
Various lesions of the respiratory tract are observed in 50% of patients. Dry throat, scratchy and scratching, dry cough and shortness of breath are the most frequent complaints.
When Sjogren’s disease is marked vascular lesion. Prickly bloody eruptions often appear on the skin of the legs, but over time they spread higher and can be found on the skin of the thighs, buttocks, and abdomen. The rash is accompanied by itching, painful burning and fever of the skin in the affected area.
Affection of the nervous system with impaired sensitivity for “socks” and “glove” types, neuritis of the facial and trigeminal nerves is observed in a third of patients.
In one third of patients allergic reactions are observed, more often – to antibiotics, sulfonamides, Novocain, preparations of group B, and also to chemical agents (washing powders, etc.) and foodstuffs.
The most informative laboratory indicators for Sjogren’s disease are high ESR , a decrease in the number of leukocytes, hypergammaglobulinemia (80–70%), the presence of antinuclear and rheumatoid factors (90–100%), as well as antibodies to soluble nuclear antigens SS-A / Ro and SS- B / La (60–100%). Cryoglobulins are found in a third of patients .
In polyclinic conditions, it is advisable to take into account various combinations of the following features:
- joint damage;
- inflammation of the parotid glands and a gradual increase in the parotid glands;
- dryness of the oral mucosa (nasopharynx) and the rapid development of multiple, mostly cervical, dental caries;
- recurrent chronic conjunctivitis;
- Raynaud’s syndrome;
- persistent increase in ESR (over 30 mm / h);
- hypergammaglobulinemia (over 20%);
- the presence of rheumatoid factor in the blood (titer more than 1: 80).
Although none of these signs, taken separately, is specific for Sjogren’s disease , the presence of four signs and more makes it possible to suspect in 80–70% of cases and further to confirm the diagnosis with the help of special research methods.
A differential diagnosis for Sjogren’s disease is performed with rheumatoid arthritis , systemic lupus erythematosus , autoimmune diseases of the liver and biliary tract in combination with Sjogren’s syndrome .
The main place in the treatment of Sjogren’s disease belongs to hormones and cytostatic immunosuppressants ( chlorbutin , cyclophosphamide ).
In the initial stage of the disease in the absence of signs of systemic manifestations and moderate violations of laboratory parameters, it is advisable long-term treatment with prednisone in small doses (5-10 mg / day).
In severe and late stages of Sjogren’s disease in the absence of signs of systemic manifestations, prednisone (5–10 mg / day) and chlorbutin (2–4 mg / day) should be prescribed, followed by prolonged, for several years, maintenance doses of prednisolone (5 mg / day) and hlorbutina (6-14 mg / week).
Such a scheme can be used to treat patients and in the initial stages of the disease in the presence of marked violations of laboratory parameters of the activity of the process, as well as cryoglobulinemia without clear signs of systemic manifestations.
Pulse therapy with high doses of prednisolone and cyclophosphane (1000 mg of 6-methylprednisolone intravenously daily for three consecutive days and a single intravenous administration of 1000 mg of cyclophosphamide ) followed by a transfer to moderate doses of prednisolone (30-40 mg / day) and cytostatics ( chlorbutin 4- 6 mg / day or cyclophosphamide 200 mg intramuscularly 1-2 times per week) in the absence of effects on the liver is the most effective treatment for patients with severe systemic manifestations of disease Sjogren usually well tolerated and n It allows one to avoid many of the complications associated with prolonged use of high doses of prednisone and cytotoxic drugs.
Extracorporeal treatments (hemosorbtion, krioadsorbtsiya, plasmapheresis, a double plasma filtration) in combination with pulse therapy is most effective in the treatment of disease Sjogren with necrotizing vasculitis, glomerulonephritis, polyneuritis, mielopoliradikulonevritom, cerebrovasculitises caused cryoglobulinemia.
Local therapy for eye damage is aimed at eliminating dryness, preventing secondary infection. Dry eye is an indication for the appointment of artificial tears. The frequency of use of drugs depends on the severity of damage to the organ of vision and varies from 3 to 10 times a day.
In order to protect the cornea, therapeutic soft contact lenses are used. For the prevention of secondary infection using solutions furatsilina (in a dilution of 1: 5000), 0.25% solution of chloramphenicol, ciprofloxacin, etc.
Therapy of chronic inflammation of the salivary glands is aimed at overcoming dryness, strengthening the walls of the ducts of the salivary glands, preventing exacerbations, improving the restoration of the epithelium of the oral mucosa and combating secondary infection.
In order to normalize nutrition and secretion of the salivary glands, novocaine blockades are used. In cases of exacerbation of chronic parotitis (and for the prevention of its recurrence), applications of 10–30% of Dimexide are used . In cases of development of purulent parotitis, antibiotics are injected into the ducts of the salivary glands and antifungal drugs are prescribed topically ( nystatin , levorin , nystatin ointment). To reduce the permeability of the ducts, calcium preparations are injected intravenously or intramuscularly.
It is possible to accelerate the healing of the oral mucosa and red border of the lips in case of erosions and cracks with the help of rose hip and sea-buckthorn oils, solkoserilovoy and methyluracil ointments, as well as by treating the oral mucosa with ENCAD (active derivatives of nucleic acids). Decamina caramel also has antibacterial properties.
With dry nasal mucosa, frequent applications of isotonic sodium chloride solution are used (with the help of turunda).
Vaginal dryness is reduced by using potassium-iodine jelly.
When the trachea and bronchi are dry, a long-term intake of Bromhexine is recommended (8–16 mg 3 times a day for 2–3 months).
If there are signs of chronic gastritis with secretory insufficiency, replacement therapy (hydrochloric acid, natural gastric juice, pepsidil ) is carried out for a long time.
Pancreatic insufficiency requires taking enzymes: panzinorm , Creon , Festal, courses of 2–3 months or permanently, and treatment with solcoseryl (2 ml intramuscularly 2 times a day for 15–30 days).
The prognosis for life is favorable. With timely treatment, it is possible to slow the progression of the disease, to restore the working capacity of patients. With a late start of treatment, severe manifestations of the disease usually develop quickly, and the patient becomes invalid.