Overview of Sjögren's Syndrome

Sjögren's Syndrome is a chronic autoimmune disease characterized primarily by immune-mediated destruction of the lacrimal and salivary glands, leading to decreased tear and saliva production. The disease may be accompanied by systemic symptoms, and in severe cases, can affect internal organ function, impacting patients' quality of life. Currently, there is no cure, but with proper treatment and daily management, symptoms can be effectively alleviated and complications prevented.

This condition is most common in middle-aged women, with a gender ratio of approximately 1:9, often misdiagnosed as aging or dry eye syndrome. Recent studies suggest that Sjögren's Syndrome may be associated with specific genetic profiles and environmental triggers. Early diagnosis is crucial for delaying disease progression. Patients should undergo regular monitoring of liver and kidney function and blood markers to prevent organ damage.

Causes and Risk Factors

The pathogenesis of Sjögren's Syndrome involves complex autoimmune abnormalities. Normally, immune cells should recognize and attack foreign pathogens, but in affected individuals, T cells and B cells mistakenly attack the tissues of the lacrimal and salivary glands, leading to inflammation and glandular atrophy. This process may be related to genetic susceptibility; studies indicate that certain human leukocyte antigen (HLA-DR3 and HLA-DQ) genes increase the risk of developing the disease.

Genetic and Environmental Interactions

Environmental triggers are considered significant factors in disease onset. Viral infections (such as Epstein-Barr virus), hormonal changes (such as menopause-related fluctuations), or prolonged exposure to chemicals may activate genetic immune deficiencies. Research shows that individuals with a history of rheumatoid arthritis or systemic lupus erythematosus have a 3-5 times higher incidence of Sjögren's Syndrome than the general population.

Risk Population Analysis

  • Age: Women over 40 have an incidence rate as high as 90%
  • Genetic predisposition: First-degree relatives with autoimmune diseases have a 2-4 times increased risk
  • Environmental exposure: Those who have undergone radiation or chemotherapy

Symptoms

The symptoms of Sjögren's Syndrome can be divided into "exocrine gland symptoms" and "systemic manifestations." The three main symptoms include: persistent dry mouth leading to swallowing difficulties, dry eyes requiring frequent artificial tears, and decreased saliva causing increased dental caries. Some patients may also experience mucosal damage such as skin cracking or nasal bleeding.

Systemic Effects

About 30% of patients experience systemic symptoms such as joint pain and fatigue. Severe cases may involve lung fibrosis, renal tubular acidosis, and other organ involvement. Neurological symptoms, such as peripheral neuropathy causing limb numbness, may also occur. Notably, the severity of symptoms does not always correlate directly with the extent of glandular damage; diagnosis should be confirmed through objective examinations.

Diagnosis

Diagnosis of Sjögren's Syndrome requires an integration of clinical symptoms and objective test results. The diagnostic criteria include the 2016 EULAR/ACR classification system, which combines symptom indicators with biological markers. Common tests include: Schirmer's test for tear production, salivary gland imaging such as sialography, and blood tests for anti-SSA/Ro and anti-SSB/La antibodies.

Differential Diagnosis Process

Physicians need to exclude drug side effects (such as antihistamines) and diseases with similar symptoms, such as primary dry eye syndrome or drug-induced xerostomia. For suspected cases, further procedures like lip gland biopsy may be performed to observe lymphocytic infiltration. Approximately 15% of patients may develop lymphoma, so regular monitoring of serum monoclonal proteins is necessary to exclude malignant transformation.

Treatment Options

The goals of treatment are to relieve symptoms, delay organ damage, and manage systemic complications. For dry mouth, salivary stimulants such as cevimeline can be used. Severe dry eyes may require autologous serum eye drops or punctal occlusion surgery. Immunomodulatory therapy, with first-line drugs like hydroxychloroquine, can reduce inflammation and has a favorable safety profile.

Emerging Treatment Strategies

  • Biologics: Rituximab targets B cell hyperactivation and is suitable for severe cases
  • Immunosuppressants: Mycophenolate mofetil for internal organ involvement
  • Supportive therapies: Sugar-free chewing gum to stimulate saliva, humidifiers to improve environmental dryness

Prevention

While Sjögren's Syndrome cannot be completely prevented, the risk of complications can be reduced by: regular dental check-ups to prevent cavities, using artificial tears and saliva substitutes to relieve symptoms. Patients are advised to quit smoking to reduce salivary gland damage and avoid antihistamines that worsen dry mouth.

Long-term Health Management

It is recommended to monitor antinuclear antibodies and complement levels every six months to assess disease activity. Patients with concomitant autoimmune diseases should pay particular attention to cardiovascular risks, with regular checks of blood lipids and blood pressure. Nutritionists suggest a high-fiber diet and avoiding dry foods to ease swallowing difficulties.

When Should You See a Doctor?

If persistent dry mouth and eye symptoms last longer than three months, and are accompanied by unexplained weight loss, joint swelling, skin purpura, or signs of decreased urination indicating renal dysfunction, immediate medical attention is necessary. If symptoms severely impact eating or sleep, autoimmune-related tests should be performed promptly.

Emergency Indicators

  • Sudden vision loss with corneal ulceration
  • Difficulty swallowing leading to dehydration or malnutrition
  • Fever with abnormal liver function tests

 

Frequently Asked Questions

How can I effectively alleviate the impact of dry mouth symptoms on daily life?

Dry mouth is a typical symptom of Sjögren's Syndrome. It can be alleviated by increasing fluid intake, using artificial saliva gels or sprays. Avoid caffeine and alcohol, and use humidifiers to maintain environmental moisture. Chewing sugar-free gum regularly can also stimulate saliva secretion and improve swallowing or speech difficulties.

Do Sjögren's patients need regular salivary gland biopsies?

Salivary gland biopsy (lip biopsy) is an important traditional diagnostic indicator for Sjögren's Syndrome, but most diagnoses nowadays rely on serological markers and symptom assessments. Repeat invasive procedures are generally unnecessary unless symptoms are unclear or initial results are inconclusive.

What is the role of biologics in the treatment of Sjögren's Syndrome?

Biologics such as anti-CD20 monoclonal antibodies (like rituximab) may be effective for patients with severe autoimmune activity, helping to slow glandular inflammation. Treatment should be based on objective assessment of disease severity, as there is an increased risk of infection, and should be used under medical supervision after individual evaluation.

How can I prevent oral infections or cavities caused by Sjögren's?

The dry environment favors plaque accumulation and fungal infections. It is recommended to use fluoride mouthwash daily and have dental check-ups every six months. If oral fissures or ulcers appear, antifungal ointments or medications should be used promptly to prevent severe infections.

How does fatigue in Sjögren's patients differ from general tiredness, and how can it be improved?

Fatigue associated with Sjögren's often accompanies chronic inflammation and persists for weeks, not improving significantly with rest. Rhythmical activity scheduling, mild aerobic exercises like walking, and cognitive-behavioral therapy can help manage and reduce fatigue symptoms effectively.

Sjögren's Syndrome