Medical expert of the article
New publications
Sjögren's syndrome
Last reviewed: 12.07.2025
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Sjogren's syndrome is a relatively common autoimmune disease of connective tissue, predominantly affecting middle-aged women and developing in approximately 30% of patients with such autoimmune pathologies as RA, SLE, scleroderma, vasculitis, mixed connective tissue disease, Hashimoto's thyroiditis, primary biliary cirrhosis and autoimmune hepatitis. Genetic determinants of the disease have been identified (in particular, HLA-DR3 antigens in Caucasians with primary Sjogren's syndrome).
Sjogren's syndrome may be primary or secondary, caused by other autoimmune diseases; at the same time, against the background of Sjogren's syndrome, arthritis may develop, resembling rheumatoid arthritis, as well as damage to various exocrine glands and other organs. Specific symptoms of Sjogren's syndrome: damage to the eyes, oral cavity and salivary glands, detection of autoantibodies and the results of histopathological examination are the basis for recognizing the disease. Treatment is symptomatic.
[ Causes of Sjogren's syndrome
Infiltration of the parenchyma of the salivary, lacrimal and other exocrine glands by CD4 + T-lymphocytes with a small number of B-lymphocytes occurs. T-lymphocytes produce inflammatory cytokines (including interleukin-2, gamma-interferon). Cells of the salivary ducts are also capable of producing cytokines that damage the excretory ducts. Atrophy of the epithelium of the lacrimal glands leads to dryness of the cornea and conjunctiva (siccated keratoconjunctivitis). Lymphocytic infiltration and proliferation of parotid gland duct cells cause narrowing of their lumen, and in some cases, the formation of compact cellular structures called myoepithelial islands. Dryness, atrophy of the mucous membrane and submucosal layer of the gastrointestinal tract and their diffuse infiltration with plasma cells and lymphocytes can lead to the development of corresponding symptoms (eg, dysphagia).
Symptoms of Sjogren's syndrome
Often, this disease initially affects the eyes and oral cavity; sometimes these symptoms of Sjogren's syndrome are the only ones. In severe cases, severe damage to the cornea develops with detachment of fragments of its epithelium (keratitis filiformis), which can lead to deterioration of vision. Decreased salivation (xerostomia) leads to impaired chewing, swallowing, secondary candidal infection, damage to the teeth and the formation of salivary duct stones. In addition, such symptoms of Sjogren's syndrome as: decreased ability to perceive smell and taste. Dry skin, mucous membranes of the nose, larynx, pharynx, bronchi and vagina may also develop. Dryness of the respiratory tract can lead to the development of cough and lung infections. The development of alopecia is also noted. A third of patients have an enlarged parotid salivary gland, which usually has a dense consistency, an even contour and is somewhat painful. With chronic mumps, the pain in the parotid gland decreases.
Arthritis develops in approximately one third of patients and is similar to that seen in patients with rheumatoid arthritis.
Other symptoms of Sjogren's syndrome may also be observed: generalized lymphadenopathy, Raynaud's phenomenon, lung parenchyma involvement (often, but only rarely serious), vasculitis (rarely with peripheral nerve and CNS involvement or with the development of skin rashes, including purpura), glomerulonephritis or multiple mononeuritis. If the kidneys are affected, tubular acidosis, impaired concentration function, interstitial nephritis and kidney stones may develop. The incidence of pseudolymphomas, malignant, including non-Hodgkin's lymphomas and Waldenstrom's macroglobulinemia in patients with Sjogren's syndrome is 40 times higher than in healthy individuals. This circumstance requires careful monitoring of these conditions. It is also possible to develop chronic diseases of the hepatobiliary system, pancreatitis (the tissue of the exocrine part of the pancreas is similar to the salivary glands), and fibrinous pericarditis.
Diagnosis of Sjögren's syndrome
Sjogren's syndrome should be suspected in a patient with excoriations, dry eyes and mouth, enlarged salivary glands, purpura, and tubular acidosis. Such patients require additional examination, including examination of the eyes, salivary glands, and serologic tests. Diagnosis is based on 6 criteria: changes in the eyes, mouth, changes in ophthalmologic examination, damage to the salivary glands, the presence of autoantibodies, and characteristic histologic changes. The diagnosis is probable if 3 or more criteria are met (including objective ones) and reliable if 4 or more criteria are met.
Signs of xerophthalmia include dry eyes for at least 3 months or the use of artificial tears at least 3 times a day. Dry eyes may also be confirmed by a slit lamp examination. Xerostomia is diagnosed by the presence of enlarged salivary glands, daily episodes of dry mouth for at least 3 months, and the need for daily fluids to aid swallowing.
The Schirmer test is used to assess the severity of dry eyes. It measures the amount of tear fluid secreted within 5 minutes after irritation by placing a strip of filter paper under the lower eyelid. In young people, the length of the moistened portion of the strip is normally 15 mm. In most patients with Sjogren's syndrome, this figure is less than 5 mm, although approximately 15% may have false-positive reactions and another 15% may have false-negative reactions. A highly specific test is the staining of the eyes when instilling eye drops containing a solution of rose bengal or lissamine green. When examined under a slit lamp, a fluorescent tear film breakup time of less than 10 seconds supports this diagnosis.
Salivary gland involvement is confirmed by abnormally low saliva production (less than 1.5 ml over 15 minutes), which is assessed by direct recording, sialography, or salivary gland scintigraphy, although these studies are used less frequently.
Serologic criteria have limited sensitivity and specificity and include antibodies to the Sjogren's syndrome antigen (Ro/SS-A) or to nuclear antigens (designated 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 increased ESR, 33% have anemia, and more than 25% have leukopenia.
If the diagnosis is unclear, a biopsy of the minor salivary glands of the buccal mucosa is necessary. Histological changes include large accumulations of lymphocytes with atrophy of the acinar tissue.
Who to contact?
Treatment of Sjögren's syndrome
Pathogenetic treatment of Sjogren's syndrome has not been developed to date. In case of dry eyes, special eye drops should be used - artificial tears, which are sold without a prescription and are instilled 4 times a day or as needed. In case of dry skin and vagina, lubricants are used.
In case of dry oral mucosa, it is useful to constantly drink small sips of liquid throughout the day, chew sugar-free gum, and use artificial saliva substitutes containing carboxymethylcellulose in the form of mouthwash. In addition, medications that reduce salivation (antihistamines, antidepressants, anticholinergics) should be excluded. Thorough oral hygiene and regular dental check-ups are necessary. Any stones that form should be removed immediately without damaging the salivary gland tissue. Pain caused by sudden enlargement of the salivary gland is best relieved by warm compresses and analgesics. Treatment of Sjogren's syndrome with pilocarpine (orally 5 mg 3-4 times a day) or cevimeline hydrochloride (30 mg) can stimulate salivation, but these medications are contraindicated in bronchospasm and closed-angle glaucoma.
In some cases, when signs of connective tissue involvement appear (e.g., when severe vasculitis or internal organ involvement develops), treatment of Sjögren's syndrome involves glucocorticoids (e.g., prednisolone, 1 mg/kg orally once daily) or cyclophosphamide (orally 5 mg/kg once daily). Arthralgias respond well to treatment with hydroxychloroquine (orally 200-400 mg once daily).
What is the prognosis for Sjogren's syndrome?
Sjogren's syndrome is a chronic disease, death may occur as a result of lung infection and, more often, as a result of renal failure or lymphoma. Its association with other connective tissue pathology worsens the prognosis.