Sjogren's Syndrome
Last reviewed: 23.04.2024
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Sjogren's syndrome is a relatively frequent autoimmune connective tissue disease that mainly affects middle-aged women and develops in approximately 30% of patients with autoimmune pathology such as RA, SLE, scleroderma, vasculitis, mixed connective tissue disease, Hashimoto's thyroiditis, primary biliary cirrhosis and autoimmune hepatitis . Identified genetic determinants of the disease (in particular, antigens HLA-DR3 in the representatives of the European race in the primary Sjögren's syndrome).
Sjogren's syndrome may be primary or secondary, due to other autoimmune diseases; at the same time, against the background of Sjogren's syndrome, development of arthritis resembling rheumatoid arthritis is possible, as well as the defeat of various exocrine glands and other organs. Specific symptoms of Sjogren's syndrome: lesions of the eyes, oral cavity and salivary glands, the detection of autoantibodies and the results of histopathological research are the basis for the recognition of the disease. Treatment is symptomatic.
[Causes of Sjogren's Syndrome
There is infiltration of parenchyma of salivary, lacrimal and other exocrine glands CD4 + T-lymphocytes with a small number of B-lymphocytes. T-lymphocytes produce inflammatory cytokines (including interleukin-2, gamma-interferon). Salivary duct cells are also capable of producing cytokines that damage excretory pathways. Atrophy of the epithelium of lacrimal glands leads to dryness of the cornea and conjunctiva (dry keratoconjunctivitis). Lymphocytic infiltration and proliferation of parotid duct cells cause narrowing of their lumen, and in some cases - the formation of compact cellular structures called myoepithelial islets. Dryness, atrophy of the mucous membrane and the submucosal layer of the gastrointestinal tract and their diffuse infiltration by plasma cells and lymphocytes can lead to the development of appropriate symptoms (eg, dysphagia).
Symptoms of Sjogren's Syndrome
Often, this disease initially marked by damage to the eyes and mouth; sometimes these symptoms of Szignren's syndrome are the only ones. In severe cases, a severe corneal lesion develops with the detachment of fragments of its epithelium (keratitis filiformis), which can lead to impaired vision. Reduction of salivation (xerostomia) leads to a violation of chewing, swallowing, secondary candidiasis infection, tooth damage and the formation of concrements of the salivary ducts. In addition, there are possible symptoms of Sjogren's syndrome, such as: a decrease in the ability to perceive smell and taste. It is also possible to develop dry skin, mucous nasal, larynx, pharynx, bronchi and vagina. Dryness of the respiratory tract can lead to the development of cough and lung infections. There is also the development of alopecia. A third of patients have an increase in the parotid salivary gland, which usually has a dense consistency, an even contour, and is somewhat painful. With chronic parotitis soreness of the parotid gland decreases.
Arthritis develops in about a third of patients and resembles those in patients with rheumatoid arthritis.
Other symptoms of Sjögren's syndrome may also be present: generalized lymphadenopathy, Raynaud's phenomenon, lung parenchymal involvement (often, but only rarely), vasculitis (in rare cases, peripheral nerves and CNS, or skin rash, including purpura) glomerulonephritis or multiple mononeuritis. With kidney damage, it is possible to develop tubular acidosis, a violation of the concentration function, interstitial nephritis and the formation of kidney stones. The frequency of pseudolymph, malignant, including non-Hodgkin's lymphomas and Waldenstrem macroglobulinemia, is 40 times higher in patients with Sjogren's syndrome than in healthy ones. This circumstance requires careful monitoring of these conditions. It is also possible to develop chronic diseases of the hepatobiliary system, pancreatitis (exocrine pancreas tissue resembles salivary glands), fibrinous pericarditis.
Diagnosis of Sjogren's Syndrome
Sjögren's syndrome should be suspected in a patient who has excoriation, dry eyes and mouth, increased salivary glands, purpura and tubular acidosis. Such patients need an additional examination, including eye examination, salivary glands and serological tests. Diagnosis is based on 6 criteria: the presence of changes on the part of the eyes, oral cavity, changes in ophthalmological examination, lesion of the salivary glands, the presence of autoantibodies and characteristic histological changes. Diagnosis is likely if there are 3 or more criteria (including objective criteria) and are reliable when 4 or more criteria are met.
Symptoms of xerophthalmia are: dry eyes for at least 3 months or use artificial tears at least 3 times a day. It is also possible to confirm the dryness of the eyes during examination in the light of the slit lamp. Xerostomia is diagnosed with salivary gland enlargement, daily dry mouth episodes for at least 3 months, daily fluid use to facilitate swallowing.
To assess the severity of the eyes, the Schirmer test is used, in which the amount of tear fluid released within 5 minutes after irritation is estimated by placing a strip of filter paper under the lower eyelid. At a young age, the length of the moistened part of the strip is normally 15 mm. In most patients with Sjogren's syndrome, this is less than 5 mm, although approximately 15% may have false positive and 15% have false-negative reactions. A highly specific test is the coloration of the eyes when instilled with eye drops containing a solution of Bengal pink or lissamine green. When examining in the light of a slit lamp in favor of this diagnosis indicates the time of rupture of a fluorescent tear film less than 10 seconds.
Salivary gland lesion is confirmed by abnormally low production of saliva (less than 1.5 ml for 15 minutes), which is estimated by direct recording, sialography or scintigraphy of salivary glands, although these studies are less commonly used.
Serological criteria have limited sensitivity and specificity and include the detection of antibodies to the antigen of Sjogren's syndrome (Ro / SS-A) or to nuclear antigens (referred to as La or SS-B), antinuclear antibodies or antibodies against gamma globulin. Rheumatoid factor is present in the serum of more than 70% of patients, 70% have an increase in ESR, 33% have anemia and more than 25% have leukopenia.
With an unclear diagnosis, it is necessary to perform a biopsy of small salivary glands of the mucous cheek. Histological changes include large accumulations of lymphocytes with atrophy of acinar tissue.
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Treatment of Sjogren's Syndrome
Pathogenetic treatment of Sjögren's syndrome has not been developed to date. If you dry your eyes, you should use special eye drops - artificial tears that are released without a prescription and buried 4 times a day or as needed. When the skin and vagina dry, lubricants are used.
When the oral mucosa is dry, it is useful to drink a small sip during the day, chew the sugar-free chewing gum and use artificial substitutes for saliva containing carboxymethyl cellulose in the form of a mouthwash. In addition, drugs that reduce salivation (antihistamines, antidepressants, anticholinergics) should be excluded. Careful oral hygiene and regular supervision at the dentist are necessary. Formed concrements must immediately be removed without damaging the tissue of the salivary gland. The pain syndrome caused by the sudden increase in the salivary gland is best suppressed by warm compresses and analgesics. Treatment of Sjogren's syndrome with pilocarpine (inside 5 mg 3-4 times a day) or tsevimelin hydrochloride (30 mg) may stimulate salivation, but these drugs are contraindicated in bronchospasm and angle-closure glaucoma.
In some cases, when signs of involvement in the process of connective tissue (for example, in the development of severe vasculitis or injury of internal organs), the treatment of Sjogren's syndrome consists in the appointment of glucocorticoids (for example, prednisolone, 1 mg / kg orally once a day) or cyclophosphamide (orally 5 mg / kg 1 time per day). Arthralgia respond well to the treatment with hydroxychloroquine (orally 200-400 mg once a day).
What is the prognosis of Sjogren's syndrome?
Sjogren's syndrome is characterized by a chronic course, death can result from infection of the lungs and, more often, due to renal failure or lymphoma. Its association with other pathology of connective tissue worsens the prognosis.