Sjogren's disease - a systemic autoimmune disease, referring to diseases of the connective tissue; It characterized by lesions of many secreting glands, mainly salivary and lacrimal.
Isolated as Sjogren's syndrome (loss of lacrimal and salivary glands) accompanying rheumatoid arthritis, Diffuse diseases of connective tissue diseases of the biliary system and other autoimmune diseases.
Sjogren's disease is the most common disease among the diseases of the connective tissue and is found in women 10-25 times more frequently than men, usually between the ages of 20-60 years, much less seen in children.
The causes of the disease are unknown. Most researchers consider Sjogren's disease as a result of immunopathological reactions to viral infection, presumably retroviral.
Manifestations of Sjogren's syndrome
Symptoms of Sjogren's disease can be divided into ferrous and vnezhelezistye.
Glandular manifestations caused by the defeat of secreting glands, characterized mainly by a decrease in their function.
A permanent sign of the defeat of the lacrimal glands in Sjogren's syndrome is an eye disease associated with decreased secretion of tear fluid. Patients complain of a burning sensation, "scratches" and "sand" in the eyes. Often marked itching eyelids, redness, congestion in the corners of the viscous white discharge. Later appear photophobia, ophthalmic narrowing slots, visual acuity decreases. The increase in the lacrimal glands in Sjogren's syndrome is rare.
The second mandatory and constant feature of Sjogren's syndrome is a defeat of the salivary glands with the development of chronic inflammation. It is characterized by dry mouth and salivary gland enlargement.
Often before the appearance of these signs are noted dry red border, perleches, stomatitisIncrease in nearby lymph nodes, multiple (usually cervical) caries teeth. One-third of patients there is a gradual increase in the parotid glands, which leads to characteristic changes in facial contours, are described in the literature as "snout hamster" or "muzzle chipmunk".
In the initial stage of the disease xerostomia occurs only during exercise and excitement.
In severe stage of dryness in the mouth becomes constant, followed by the need to wash down the dry food, the desire to moisten your mouth while talking. Oral mucosa becomes bright pink, easily injured. Free little saliva, it frothy or sticky. Tongue dry. Lips covered with crusts, the phenomena of inflammation, may join a secondary infection, including fungal and viral. Characteristic multiple cervical caries.
Late stage appears sharp dryness of the mouth, inability to speak, swallow food, it is not squeezed liquid. Lips dry these patients, in cracks, oral mucosa with signs of keratinization, tongue folded free saliva in the oral cavity is not defined.
Dryness nasopharynx to form dry crusts in the nose, in the lumen of the auditory tubes can lead to temporary deafness and Development otitis. Dryness of the throat and vocal cords causes hoarseness.
Frequent complications are secondary infections: sinusitis, Recurrent tracheobronchitis and pneumonia. The defeat of the glands of the external genitalia occurs in about 1/3 of patients with Sjogren's syndrome. Vaginal mucosa reddened, dry, often patients concerned about burning pain and itching.
A frequent symptom of the disease Sjogren's is dry skin. Sweating may be reduced.
Swallowing disorder caused by the presence of mucous dryness. Many patients develop chronic atrophic gastritis with severe secretory failure, clinically manifesting the weight and discomfort in the epigastric region after eating, belching air, nausea, decreased appetite. Less frequent pain in the epigastric region.
There is a direct relationship between the degree of dryness and the oppression of the secretory function of the stomach. biliary tract disease (cholecystitis) And the liver (hepatitis) Is observed in most patients. There are complaints of heaviness and pain in the right upper quadrant, the bitterness in the mouth, nausea, poor tolerance of fatty foods.
Involvement in pancreatic process (Pancreatitis) manifested by pain and digestive problems.
Vnezhelezistye manifestations of Sjögren's disease are very diverse, are systemic in nature. Joint pain, slight stiffness in the morning. Signs of inflammation of the muscles (muscle aches, mild muscle weakness, a slight increase in blood creatine phosphokinase) are observed in 5-10% of patients.
Most patients with Sjogren's syndrome there is an increase of submandibular, cervical, occipital, supraclavicular lymph nodes, and 1/3 patients with lymph node enlargement is common in nature. In the latter case often revealed an enlarged liver.
Various lesions of the respiratory tract were observed in 50% of patients. Dry throat, sore and scarring, dry cough and shortness of breath are the most frequent complaints.
When Sjogren's syndrome is marked vascular disease. Melkotochechnye bloody rashes often appear on the skin of shins, but eventually spread above and can be detected on the skin of the thighs, buttocks and abdomen. Rash accompanied by itching, burning and painful rise in temperature of the skin in the affected area.
The defeat of the nervous system with sensory disorders of the type "socks" and "gloves", neuritis of the facial and trigeminal nerves observed in one third of patients.
One-third of patients are allergic, more often - antibiotics, sulfonamides, procaine, Group B drugs, as well as chemical means (detergents, etc.), And foods.
Diagnostics
The most informative laboratory parameters with Sjogren's syndrome are high ESR, Reducing the number of leukocytes, hypergammaglobulinemia (80-70%), the presence of antinuclear and rheumatoid factors (90-100%), as well as antibodies to soluble nuclear antigen SS-A / Ro and SS-B / La (60-100%). One-third of patients are found cryoglobulins.
The polyclinic conditions it is advisable to take into account the various combinations of the following characteristics:
- joint disease;
- inflammation of the parotid salivary glands and a gradual increase in the parotid glands;
- dryness of the oral mucosa (the nasopharynx) and the rapid development of multiple, preferably cervical, dental caries;
- chronic recurrent conjunctivitis;
- Raynaud's syndrome;
- persistent increase in ESR (more than 30 mm / h);
- hypergammaglobulinemia (over 20%);
- the presence of rheumatoid factor in the blood (titer greater than 1: 80).
While none of these features, taken in isolation, is not specific for Sjögren's disease, the presence of the four criteria and more allows 80-70% of cases of suspected and later with the help of special methods of investigation to confirm the diagnosis.
Differential diagnosis of Sjogren's disease is carried out rheumatoid arthritis, systemic lupus erythematosusAutoimmune diseases of the liver and biliary tract, combined with Sjögren's syndrome.
Sjogren's disease treatment
The main place in the treatment of Sjogren's disease belongs to the hormones and cytotoxic immunosuppressants (hlorbutin, cyclophosphamide).
In the initial stage of the disease in the absence of systemic manifestations and moderate irregularities laboratory indicators should long-term treatment with prednisone in small doses (5-10 mg / day).
The expressed and late stages of Sjogren's syndrome in the absence of systemic manifestations necessary appointment prednisolone (5-10 mg / day) and chlorambucil (2-4 mg / day) followed by a prolonged, for several years, receiving maintenance doses of prednisolone (5 mg / day), and chlorambucil (6-14 mg / week).
Such a circuit may be used to treat patients in the early stages of the disease in the presence of the expressed disorders laboratory parameters of process activity and cryoglobulinemia without clear signs of systemic manifestations.
Pulse therapy with high doses of prednisone and cyclophosphamide (1000 mg of 6-methylprednisolone intravenously daily for three consecutive days, and single intravenous cyclophosphamide administering 1000 mg), followed by transfer to a moderate dose of prednisolone (30-40 mg / day) and cytostatics (hlorbutin 4-6 mg / day or 200 mg cyclophosphamide intramuscularly, 1-2 times per week) in the absence of exposure to the liver was the most effective treatment for patients with severe systemic manifestations Sjogren's disease, is generally well tolerated by patients and avoids many of the complications associated with prolonged use of high doses of prednisone and cytostatics.
Extracorporeal therapies (hemosorbtion, krioadsorbtsiya, plasmapheresis, plasma double filtration) in combination with pulse therapy is most effective in patients with Sjogren's disease necrotizing vasculitis, glomerulonephritis, Polyneuritis, mielopoliradikulonevritom, cerebrovasculitises caused by cryoglobulinemia.
Local therapy for eye disease is aimed at eliminating dryness, to prevent secondary infection. Dry eye is an indication for artificial tears. Frequency of drugs depends on the severity of the organ of vision and ranges from 3 to 10 times a day.
With therapeutic soft contact lenses are used purpose corneal protection. For the prevention of secondary infection are used furatsilina solutions (diluted 1: 5000), 0.25% solution of chloramphenicol, ciprofloxacin, and others.
Therapy of chronic inflammation of the salivary glands is aimed at overcoming dryness, strengthening the walls of the salivary ducts glands, prevention of exacerbations, improve oral mucosal epithelium recovery mouth and fight the secondary infection.
To normalize the power and secretion of the salivary glands using procaine blockade. In cases of exacerbation of chronic parotitis (and for the prevention of its recurrence) apply appliqué Dimexidum 10-30% solution. In cases of purulent mumps in the ducts of the salivary glands is administered topically administered antibiotics and antifungal drugs (nystatin, Levorinum, nystatin ointment). To reduce the permeability ducts administered intravenously or intramuscularly calcium preparations.
Accelerate healing of the oral mucosa and the vermilion border at occurrence of erosion and cracks manages via rosehip oil and buckthorn solkoserilovoy and methyluracyl ointments, as well as by treatment of the oral mucosa ENKAD drug (active derivatives of nucleic acids). Also possesses antibacterial properties dekaminovaya caramel.
When nasal mucosa dryness used frequent applications isotonic sodium chloride solution (via turundae).
Vaginal dryness is reduced when using the potassium iodide-jelly.
When dry trachea and bronchi recommended long term administration of bromhexine (8-16 mg 3 times a day for 2-3 months).
If there are indications chronic gastritis with secretory insufficiency for long periods carried replacement therapy (hydrochloric acid, natural gastric juice, Pepcid).
Pancreatic insufficiency requires reception enzymes panzinorma, Creon, Festalum courses 2-3 month or continuously, as is the treatment solkoserilom (2 ml intramuscularly 2 times a day for 15-30 days).
Forecast
Weather favorable for life. When time begun treatment manages to slow the progression of the disease, patients recover the ability to work. In case of late start of treatment is usually rapidly develop severe manifestations of the disease, the patient's disability occurs.
