Sjögren's Syndrome: Symptoms, Causes & Treatment Guide

What Is Sjögren's Syndrome?

Sjögren's syndrome is a chronic autoimmune disorder where the immune system mistakenly attacks the body's moisture-producing glands, primarily the tear and salivary glands. This results in the characteristic symptoms of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), but can also affect other organs throughout the body.

Sjögren's syndrome is named after Swedish ophthalmologist Henrik Sjögren, who first described the condition in 1933. The disease is considered one of the most common autoimmune disorders, affecting an estimated 0.5-1% of the population worldwide. It is particularly prevalent among middle-aged women, with a female-to-male ratio of approximately 9:1.

In this condition, the immune system, which normally protects the body from infections and foreign invaders, turns against the body's own tissues. White blood cells infiltrate and damage the exocrine glands, which are responsible for producing moisture. The salivary glands in the mouth and the lacrimal (tear) glands in the eyes are primarily affected, but the condition can also impact glands in the skin, respiratory tract, and genital area.

The disease can occur at any age, but diagnosis most commonly occurs between ages 40 and 60. While Sjögren's syndrome is rare in children, it can occur and may present differently than in adults. The condition tends to develop gradually, and many people experience symptoms for years before receiving a diagnosis.

Primary vs. Secondary Sjögren's Syndrome

Sjögren's syndrome is classified into two distinct types based on whether it occurs alone or in combination with other autoimmune conditions:

Primary Sjögren's syndrome occurs as a standalone autoimmune condition without any other connective tissue disease. Patients with primary Sjögren's experience dryness symptoms and may also have systemic manifestations affecting joints, lungs, kidneys, or the nervous system. This form tends to have more pronounced sicca (dryness) symptoms and is associated with specific autoantibodies (anti-SSA/Ro and anti-SSB/La).

Secondary Sjögren's syndrome develops in patients who already have another autoimmune disease. The most commonly associated conditions include rheumatoid arthritis, systemic lupus erythematosus (SLE), and systemic sclerosis. In secondary Sjögren's, the symptoms of the underlying condition often dominate the clinical picture, though dryness symptoms can still significantly impact quality of life. Treatment must address both the primary autoimmune disease and the Sjögren's-related symptoms.

What Are the Symptoms of Sjögren's Syndrome?

The primary symptoms of Sjögren's syndrome are dry eyes that burn, itch, and feel gritty, along with dry mouth that makes swallowing difficult and increases cavity risk. Many patients also experience severe fatigue, joint pain, and dryness in other areas including the skin, nose, throat, and vaginal tissues.

Sjögren's syndrome symptoms can range from mild to severe and may fluctuate over time. Many patients experience periods of increased disease activity called "flares," followed by periods of relative remission. Understanding the full spectrum of symptoms helps with early recognition and appropriate management.

The dryness symptoms are often the most noticeable and can significantly impact daily activities. However, it's important to recognize that dryness alone doesn't necessarily indicate Sjögren's syndrome, as these symptoms become more common with age and can result from various other causes. The distinguishing features of Sjögren's-related dryness are its severity, persistence, and association with other symptoms.

Dry Eyes (Keratoconjunctivitis Sicca)

Dry eyes are often one of the first symptoms patients notice. The tear glands (lacrimal glands) produce insufficient tears, or the tears produced are of poor quality and evaporate too quickly. This leads to inadequate lubrication and protection of the eye surface.

Common eye symptoms include:

• Burning and stinging sensation: A persistent feeling of irritation that may worsen throughout the day
• Gritty or sandy feeling: The sensation of having sand or foreign particles in the eyes, even when nothing is present
• Redness and inflammation: The conjunctiva (white of the eye) may appear red and irritated
• Light sensitivity (photophobia): Increased discomfort in bright light or sunlight
• Blurred vision: Particularly after sustained reading or screen use
• Excessive tearing: Paradoxically, the eyes may produce reflex tears in response to irritation
• Eye fatigue: A feeling of tired, heavy eyes, especially toward evening

Environmental factors such as air conditioning, heating, wind, smoke, and prolonged screen use can significantly worsen dry eye symptoms. Many patients find that symptoms fluctuate seasonally, often worsening during dry winter months when indoor heating reduces humidity.

Dry Mouth (Xerostomia)

Dry mouth occurs when the salivary glands produce insufficient saliva. Saliva plays crucial roles beyond moisture, helping with digestion, protecting teeth from decay, and fighting infections. The reduction in saliva can therefore have wide-ranging effects.

Dry mouth symptoms include:

• Difficulty swallowing: Especially dry foods like crackers or bread without liquids
• Changes in taste: Food may taste different or have a metallic flavor
• Difficulty speaking: Particularly for extended periods, as the mouth and throat feel parched
• Sore or cracked lips: And cracks at the corners of the mouth (angular cheilitis)
• Increased thirst: A constant need to sip water
• Burning sensation: On the tongue or inside the mouth
• Bad breath (halitosis): Due to reduced antimicrobial action of saliva
• Frequent dental cavities: Despite good oral hygiene, due to loss of saliva's protective effects

Fatigue

Fatigue is one of the most common and often most debilitating symptoms of Sjögren's syndrome. Unlike ordinary tiredness, this fatigue is profound, persistent, and not relieved by rest. Many patients describe it as overwhelming exhaustion that significantly impacts their ability to work, socialize, and perform daily activities.

The fatigue associated with Sjögren's syndrome may come in waves, with good days and bad days. Flares of disease activity often bring increased fatigue. Research suggests the fatigue is related to the chronic inflammation present in autoimmune diseases and may also be influenced by sleep disturbances caused by dryness symptoms.

Joint and Muscle Pain

Many people with Sjögren's syndrome experience musculoskeletal symptoms including joint pain (arthralgia) and muscle aches (myalgia). The joints most commonly affected are the small joints of the hands and fingers, though larger joints can also be involved.

Joint symptoms typically include:

• Morning stiffness that improves with movement
• Joint swelling, particularly in the fingers
• Pain that comes and goes in episodes
• Diffuse muscle aching, especially in the arms and legs

Unlike rheumatoid arthritis, Sjögren's syndrome typically does not cause the same degree of joint erosion and deformity. However, when secondary Sjögren's occurs alongside rheumatoid arthritis, more significant joint damage may develop.

Other Dryness Symptoms

The dryness in Sjögren's syndrome can extend beyond the eyes and mouth to affect other areas:

Dry skin: The skin may become dry, itchy, and prone to irritation. Some patients develop skin rashes, including a characteristic rash on the lower legs called purpura.

Dry nose and throat: This can lead to persistent dry cough, hoarseness, and reduced sense of smell. Nasal crusting and nosebleeds may occur.

Vaginal dryness: Women may experience dryness of vaginal tissues, leading to discomfort during intercourse and increased susceptibility to yeast infections. This symptom can be worsened by menopause-related hormonal changes.

When Should You See a Doctor for Sjögren's Symptoms?

You should see a doctor if you experience persistent dry eyes or dry mouth lasting more than 2-3 months, especially if accompanied by fatigue, joint pain, or difficulty swallowing. Early diagnosis allows for better symptom management and monitoring for potential complications.

Many of the symptoms of Sjögren's syndrome, such as dry eyes and fatigue, are common in the general population and can have many causes. However, certain patterns and combinations of symptoms warrant medical evaluation for possible Sjögren's syndrome.

Consider consulting a healthcare provider if you experience:

• Dry eyes and/or dry mouth that persist for more than 2-3 months
• Need to drink water frequently to swallow food
• Recurrent mouth infections or dental cavities despite good oral hygiene
• Persistent fatigue that is not explained by lifestyle factors
• Joint pain or swelling, particularly in the fingers
• Swollen salivary glands (in front of the ears or under the jaw)

If you already have another autoimmune condition such as rheumatoid arthritis or lupus and develop significant dryness symptoms, discuss this with your rheumatologist, as you may have developed secondary Sjögren's syndrome.

How Is Sjögren's Syndrome Diagnosed?

Diagnosis of Sjögren's syndrome involves multiple tests including blood tests for autoantibodies (anti-SSA/Ro, anti-SSB/La), the Schirmer test for tear production, salivary flow measurement, and sometimes a lip biopsy to examine minor salivary glands for characteristic inflammation.

Diagnosing Sjögren's syndrome can be challenging because its symptoms overlap with many other conditions, and there is no single definitive test. The average time from symptom onset to diagnosis is 4-7 years. Diagnosis typically involves a combination of clinical evaluation, laboratory tests, and specialized assessments following the ACR/EULAR classification criteria.

Initial Evaluation

The diagnostic process begins with a thorough medical history and physical examination. Your doctor will ask detailed questions about your symptoms, their duration, and their impact on daily life. They will also inquire about other medical conditions, family history of autoimmune diseases, and current medications, as some medications can cause dryness symptoms.

The physical examination includes checking for enlarged salivary glands, examining the mouth for signs of dryness and dental disease, and looking for other manifestations such as joint swelling or skin changes.

Blood Tests

Several blood tests help support the diagnosis:

Autoantibodies: The most specific blood tests look for anti-SSA/Ro and anti-SSB/La antibodies. Anti-SSA antibodies are present in about 60-70% of primary Sjögren's patients, while anti-SSB antibodies are found in about 40%. Having these antibodies strongly supports the diagnosis, but their absence doesn't rule it out.

Antinuclear antibodies (ANA): Present in many autoimmune conditions, ANA is positive in most Sjögren's patients but is not specific to the condition.

Rheumatoid factor: Often elevated in Sjögren's syndrome, though not specific to this condition.

Inflammatory markers: Tests such as ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein) may be elevated, indicating inflammation.

Complete blood count: May show anemia or low white blood cell counts in some patients.

Eye Tests

Eye examination by an ophthalmologist includes several assessments:

Schirmer test: A thin paper strip is placed under the lower eyelid for 5 minutes to measure tear production. Less than 5mm of wetting is considered abnormal and consistent with dry eyes.

Rose Bengal or Lissamine Green staining: Special dyes are applied to the eye surface to reveal damage to the cornea and conjunctiva caused by dryness.

Tear break-up time: Measures how quickly the tear film on the eye surface breaks up after blinking, indicating tear quality.

Salivary Gland Assessment

Tests to evaluate salivary function include:

Unstimulated salivary flow rate: You sit with your mouth slightly open over a container, allowing saliva to flow naturally for 15 minutes. Less than 0.1 mL per minute is considered abnormal.

Stimulated salivary flow rate: Similar test performed while chewing gum to stimulate saliva production.

Salivary gland imaging: Ultrasound or MRI can show characteristic changes in the salivary glands.

Lip Biopsy

A minor salivary gland biopsy from the inner lower lip can be highly informative. This outpatient procedure involves removing several tiny glands through a small incision made under local anesthesia. A pathologist examines the tissue for lymphocyte infiltration, a hallmark finding in Sjögren's syndrome.

The biopsy is particularly useful when blood tests are negative or inconclusive. A positive biopsy showing characteristic focal lymphocytic sialadenitis is one of the key criteria for diagnosis.

How Is Sjögren's Syndrome Treated?

Treatment focuses on relieving symptoms and preventing complications. Key approaches include artificial tears and lubricating eye gels for dry eyes, saliva substitutes and stimulants (like pilocarpine) for dry mouth, anti-inflammatory medications for systemic symptoms, and meticulous dental care to prevent cavities.

While there is currently no cure for Sjögren's syndrome, effective treatments can manage symptoms, prevent complications, and help maintain quality of life. Treatment is tailored to each individual based on which symptoms are most troublesome and whether systemic involvement is present.

Treatment for Dry Eyes

Managing dry eyes is essential for comfort and preventing corneal damage:

Artificial tears: These are the mainstay of treatment. Preservative-free formulations are preferred for frequent use (more than 4 times daily) as preservatives can irritate the eyes over time. Various viscosities are available, from thin, watery drops for mild symptoms to thicker gels for more severe dryness.

Lubricating ointments: Thicker preparations are used at bedtime to prevent overnight drying. They can blur vision, so they're generally not used during waking hours.

Prescription eye drops: Cyclosporine (Restasis) or lifitegrast (Xiidra) eye drops reduce inflammation on the eye surface and can improve tear production over time. Benefits typically appear after several weeks to months of consistent use.

Punctal plugs: Tiny silicone or collagen plugs can be inserted into the tear drainage ducts to keep tears on the eye surface longer. This is particularly helpful for moderate to severe dry eyes.

Treatment for Dry Mouth

Managing dry mouth helps with comfort, eating, speaking, and dental health:

Saliva substitutes: These products mimic natural saliva and can be used as needed throughout the day. Available as sprays, gels, or lozenges.

Saliva stimulants: Prescription medications such as pilocarpine (Salagen) or cevimeline (Evoxac) stimulate the remaining functional salivary gland tissue to produce more saliva. Side effects can include sweating and increased urination.

Sugar-free gum and lozenges: Chewing stimulates saliva production. Sugar-free products are essential to prevent dental decay.

Adequate hydration: Drinking water regularly throughout the day helps manage dryness. Sipping water during meals aids swallowing.

Systemic Treatments

For patients with significant systemic involvement, additional medications may be needed:

Hydroxychloroquine (Plaquenil): This antimalarial drug has immunomodulatory effects and can help with fatigue and joint pain. It requires regular eye exams to monitor for rare retinal effects.

Nonsteroidal anti-inflammatory drugs (NSAIDs): Can help manage joint and muscle pain.

Corticosteroids: May be used for significant joint inflammation or during disease flares. Low-dose oral prednisone or injections into affected joints can provide relief.

Immunosuppressive medications: For severe systemic manifestations affecting organs like the lungs, kidneys, or nervous system, medications such as methotrexate, azathioprine, or rituximab may be considered.

Dental Care

Meticulous dental care is crucial due to the greatly increased risk of cavities:

• Regular dental checkups every 3-6 months
• Fluoride treatments and prescription fluoride toothpaste
• Avoiding sugary foods and drinks
• Prompt treatment of any dental problems

Treatment for Vaginal Dryness

For women experiencing vaginal dryness:

• Water-based vaginal lubricants for comfort during intercourse
• Vaginal moisturizers used regularly
• Low-dose vaginal estrogen (vaginal tablets, cream, or ring) for postmenopausal women, if not contraindicated

What Can You Do to Manage Sjögren's Symptoms at Home?

Effective self-care includes using a humidifier at home, wearing wrap-around sunglasses outdoors, avoiding dry environments, staying well-hydrated, choosing moist foods that are easy to chew and swallow, and practicing excellent dental hygiene with fluoride products.

Many lifestyle modifications can significantly improve symptoms and quality of life. These measures complement medical treatment and can make daily activities more comfortable.

Environmental Modifications

Controlling your environment can minimize symptom triggers:

• Use humidifiers: Maintain indoor humidity between 40-60%, particularly in bedrooms and workspaces
• Avoid direct airflow: Position away from air conditioning vents, heating ducts, and fans
• Wear wrap-around sunglasses: Protects eyes from wind and bright light outdoors
• Take regular screen breaks: Follow the 20-20-20 rule when using computers
• Avoid smoke and air pollution: These irritate dry eyes and respiratory passages

Nutrition and Hydration

Dietary adjustments can ease eating difficulties:

• Drink water frequently, especially during meals
• Choose moist foods like soups, stews, and foods with sauces or gravies
• Cut food into small pieces to make chewing easier
• Avoid dry, crumbly foods that are difficult to swallow
• Limit caffeine and alcohol, which can worsen dryness
• Avoid acidic foods and drinks that can irritate a dry mouth

Oral Hygiene

Protecting your teeth is essential:

• Brush teeth after every meal with fluoride toothpaste
• Floss daily to prevent gum disease
• Use alcohol-free mouth rinses (alcohol worsens dryness)
• Consider prescription fluoride products as recommended by your dentist
• Chew sugar-free gum to stimulate saliva

Exercise and Rest

Physical activity and adequate rest help manage fatigue and joint symptoms:

• Regular gentle exercise such as swimming, walking, or cycling
• Water aerobics or pool exercises, which are particularly helpful for joint pain
• Stretching exercises to maintain flexibility
• Balance activity with rest, pacing yourself during flares
• Maintain regular sleep schedules

What Are the Complications of Sjögren's Syndrome?

Potential complications include dental decay and tooth loss from chronic dry mouth, corneal damage from dry eyes, increased infection risk, and rarely, lung or kidney problems, nerve damage, or lymphoma. Regular monitoring helps detect and address complications early.

While most people with Sjögren's syndrome manage well with treatment, the condition can lead to various complications. Understanding these risks helps with early detection and prevention.

Dental Complications

The reduced saliva flow significantly increases the risk of dental problems. Saliva normally helps neutralize acids, wash away food particles, and has antimicrobial properties. Without adequate saliva, patients may experience rapid development of cavities, even with excellent oral hygiene. Regular dental care and preventive measures are essential.

Eye Complications

Chronic dry eyes can lead to damage of the cornea (the clear front surface of the eye). Without treatment, this can result in corneal ulcers, scarring, and vision problems. Regular ophthalmology monitoring helps prevent serious eye damage.

Infections

The body's mucous membranes serve as a first line of defense against infections. Reduced moisture increases susceptibility to:

• Oral yeast infections (thrush)
• Bacterial sialadenitis (salivary gland infection)
• Vaginal yeast infections
• Respiratory infections

Systemic Complications

In some patients, Sjögren's syndrome can affect other organ systems:

Lung involvement: Can include dry cough, interstitial lung disease, or increased susceptibility to bronchitis.

Kidney involvement: May include interstitial nephritis or kidney stones.

Neurological involvement: Peripheral neuropathy causing numbness, tingling, or burning sensations in hands and feet.

Thyroid problems: Increased risk of thyroiditis (inflammation of the thyroid gland).

Lymphoma Risk

People with Sjögren's syndrome have a slightly increased risk (about 5%) of developing non-Hodgkin lymphoma, particularly B-cell lymphoma. This risk is higher in those with severe disease, persistent salivary gland swelling, low complement levels, or certain other blood test abnormalities.

What Is It Like Living with Sjögren's Syndrome?

Most people with Sjögren's syndrome lead full, active lives with proper management. Success involves developing daily routines for symptom management, regular medical follow-up, connecting with support resources, and learning to pace activities to manage fatigue effectively.

Living with Sjögren's syndrome requires adapting to daily symptom management while maintaining quality of life. With appropriate treatment and self-care strategies, most people successfully balance managing their condition with work, family, and social activities.

Daily Management

Establishing routines helps ensure consistent symptom management:

• Keep artificial tears and lip balm readily available at home, work, and in your bag
• Always carry a water bottle
• Plan regular breaks during work for eye drops and hydration
• Prepare for travel with adequate supplies

Managing Fatigue

Fatigue often requires the most significant lifestyle adjustments. Strategies include:

• Prioritizing essential activities and delegating when possible
• Planning demanding activities for times of peak energy
• Taking short rest breaks throughout the day
• Accepting that some days will be harder than others
• Communicating needs to family, friends, and employers

Regular Medical Follow-up

Ongoing medical care is important for:

• Monitoring disease activity and adjusting treatment
• Screening for complications
• Regular dental checkups (every 3-6 months)
• Annual eye examinations
• Periodic blood tests

Pregnancy Considerations

Sjögren's syndrome generally does not affect fertility or pregnancy outcomes. However, women with anti-SSA/Ro or anti-SSB/La antibodies have a small risk of having a baby with neonatal lupus or congenital heart block. Pregnancy should be planned with medical guidance, and specialized monitoring may be recommended.

Support and Resources

Connecting with others who understand the condition can be valuable:

• Patient support organizations and foundations
• Online communities and forums
• Local support groups
• Educational resources from rheumatology societies