Do you know? Open the door┋Dry mouth, dry eyes, do I have Sjögren's syndrome?

Author: Zhang Chenxi, resident physician in the Department of Rheumatology and Immunology, the First Affiliated Hospital of Xinjiang Medical University.

Review expert: Luo Li, chairman of the Clinical Immunology Branch of the Xinjiang Immunology Society; vice president of the Xinjiang Uygur Autonomous Region Immunology Society; director, professor and chief physician of the Department of Rheumatology and Immunology, the First Affiliated Hospital of Xinjiang Medical University.

In autumn and winter, when the weather is dry, many people experience dry mouth, dry eyes, or dry skin. It is generally believed that this is related to the climate and is a normal phenomenon. However, if you experience repeated dry mouth and dry eyes, especially difficulty eating, frequent tooth decay, broken and flaky teeth, and crying without tears, then you should be alert to Sjögren's syndrome!

1. What is Sjögren's syndrome?

The so-called "dryness" of Sjögren's syndrome is a chronic inflammatory autoimmune disease characterized by lymphocyte proliferation and progressive exocrine gland (tear gland, salivary gland, etc.) damage. It is unrelated to changes in weather, temperature, humidity, etc., and the discomfort cannot be relieved by moisturizing or environmental changes. The prevalence rate in the Chinese population is 0.3% to 0.7%, and the disease is more common in women aged 40 to 50 years old, and can also be seen in children. The cause of Sjögren's syndrome has not yet been clarified. Current research believes that it is related to immune dysfunction caused by genetics, endocrine hormones, infections and other factors.

2. What are the symptoms of Sjögren's syndrome?

① Local symptoms:

Dry mouth : Reduced saliva secretion and lack of salivary mucin lead to frequent drinking of water. When eating dry foods such as steamed bread, naan, and rice, water is often needed. In severe cases, eating difficulties, flaky tooth loss, and multiple rampant dental caries may occur. Some patients may experience repeated parotid gland swelling.

Dry eyes : Low tear gland secretion function leads to symptoms of dry eyes, gritty feeling, and eye congestion. In severe cases, complications such as keratoconjunctivitis sicca, corneal epithelial erosion, corneal neovascularization and ulcer formation may occur, and even corneal perforation and blindness may occur.

② System involvement:

Some patients may experience involvement of internal organs, which may be accompanied by systemic symptoms such as fatigue and fever.

Skin: mainly include dry skin, Raynaud's phenomenon (fingers and toes turn white, purple, flushed, etc. in succession in response to cold stimulation, emotional excitement, etc.), purpura of the lower limbs, skin ulcers, gangrene, depressed scars, microinfarction and other vasculitic changes.

Joints : Recurrent, chronic, symmetrical joint pain. Only about 10% of patients experience joint swelling and pain. Erosive arthritis is rare.

Respiratory system: 10%-20% of patients may develop Sjögren's syndrome-related interstitial lung lesions, the pathological types of which are often manifested as nonspecific interstitial pneumonia (NSIP), lymphocytic interstitial pneumonia (LIP) and usual interstitial pneumonia (UIP).

Digestive system: There are often symptoms of gastroesophageal reflux disease, such as acid reflux, heartburn, and throat and tracheal irritation. This is because the saliva flow is reduced and cannot naturally buffer the reflux of acidic gastric contents. Some patients have liver damage, elevated transaminases, and even jaundice, and occasionally hepatosplenomegaly. When the patient's serum alkaline phosphatase level is elevated, you should be alert to whether it is combined with primary biliary cholangitis (PBC), as the two often occur together.

Kidney: The most common disease is tubulointerstitial lesions, which lead to renal tubular acidosis. Patients often seek medical attention due to hypokalemia. Glomerulonephritis and interstitial cystitis may also occur.

Nervous system: Peripheral neuropathy is the most common, and the peripheral nerves, autonomic nerves and central nervous system can all be affected.

Blood system: Leukopenia is the most common, followed by secondary thrombocytopenia. The risk of developing lymphoma is 18.9 times that of the normal population.

3. Do dry mouth and dry eyes indicate Sjögren's syndrome?

First of all, physiological factors should be ruled out, such as dry mouth and dry eyes caused by insufficient water intake, excessive water loss in the body, eating too salty food or a lot of dry food, and dry climate.

Secondly, other pathological factors need to be ruled out. Dry lips may also occur in patients with diabetes, diabetes insipidus, pernicious anemia, asthma, oral diseases, tumor radiotherapy, and emotional instability in women with menopausal syndrome.

4. How to diagnose Sjögren's syndrome and what tests are needed

If you have the above typical symptoms, you need to go to the hospital for evaluation by a rheumatologist and have relevant examinations completed according to different clinical manifestations, such as three dry eye tests (tear secretion test, tear film breakup time, corneal fluorescence staining), salivary gland ECT, lip gland biopsy, connective tissue disease series autoantibody tests (among which anti-SSA antibodies and anti-SSB antibodies have the highest positive rates and are the most specific antibodies for diagnosing SS), and systemic involvement assessment based on the condition to further clarify the diagnosis.

5. How to treat Sjögren's syndrome

The treatment goal of Sjögren's syndrome is not only to relieve local symptoms such as dry mouth and dry eyes, but more importantly to control the body's immune dysfunction and reduce exocrine gland damage and systemic involvement.

Treatment of Sjögren's syndrome requires a specialist to develop an individualized treatment plan based on a comprehensive assessment of the disease activity and the organs involved. Do not blindly follow the trend and take medication. Currently, commonly used drugs include glucocorticoids, immunomodulators, immunosuppressants, and biological agents.

6. What is the prognosis of Sjögren's syndrome?

The overall prognosis of Sjögren's syndrome is good, especially when the disease is limited to the exocrine glands such as salivary glands, lacrimal glands, and skin mucosa. Most patients with visceral involvement can have their disease effectively controlled after active and regular treatment. Poor prognostic factors include progressive pulmonary interstitial fibrosis, central nervous system lesions, renal insufficiency, and combined malignant lymphoma.

References:

[1] Zhang Wen, Chen Zhu, Li Xiaomei, et al. Diagnosis and treatment guidelines for primary Sjögren's syndrome[J]. Chinese Journal of Internal Medicine, 2023, 62(9): 1059-1067. DOI: 10.3760/cma.j.cn112138-20221027-00797.

[2]Zero DT , Brennan MT , Daniels TE ,et al.Clinical practice guidelines for oral management of Sjgren disease[J].The Journal of the American Dental Association, 2016, 147(4):295-305.DOI:10.1016/j.adaj.2015.11.008.

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