Understanding Sjögren Syndrome and Its Effects on the Body

Written by Medicover Team and Medically Reviewed by Dr Bhavana Surapareddy , Rheumatologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Sjögren's Syndrome is an autoimmune disorder in which the immune system attacks the body's moisture-producing glands, leading to dryness in various parts of the body. This condition can be challenging to diagnose because its symptoms may overlap with other autoimmune diseases such as lupus. Sjögren's Syndrome most commonly affects the eyes and mouth but can also involve other parts of the body.

Sjögren's Syndrome primarily affects the salivary and lacrimal glands, causing dry mouth and dry eyes. However, it can also affect other parts of the body, including the joints, thyroid, kidneys, liver, lungs, skin, and nerves.

Sjögren's Syndrome can occur alone, known as primary Sjögren's Syndrome, or alongside another autoimmune disease, such as lupus or rheumatoid arthritis, known as secondary Sjögren's Syndrome.

What Are the Types of Sjögren's Syndrome?

Sjögren's Syndrome is generally classified according to whether it occurs on its own or together with another autoimmune disease.

Primary Sjögren's Syndrome: Occurs on its own without another associated autoimmune disease.
Secondary Sjögren's Syndrome: Occurs alongside another autoimmune disease, such as rheumatoid arthritis, lupus, or systemic sclerosis.

What Are the Symptoms of Sjögren's Syndrome?

The symptoms of Sjögren's Syndrome can vary widely among individuals, making it a particularly challenging condition to diagnose. Common symptoms include:

Dry Mouth (Xerostomia)

One of the hallmark symptoms of Sjögren's Syndrome is persistent dry mouth. This can lead to difficulties in speaking, swallowing, and tasting food. It can also increase the risk of dental cavities and oral infections.

Dry Eyes (Keratoconjunctivitis Sicca)

Dry eyes are another primary symptom. Patients may experience a gritty or burning sensation in the eyes, which can lead to chronic irritation and, if untreated, more serious eye complications.

Fatigue

Fatigue is a common symptom of Sjögren's Syndrome and can significantly affect quality of life. This fatigue may be persistent and can make daily activities difficult.

Joint Pain and Swelling

Many individuals with Sjögren's Syndrome experience joint pain and swelling, which may resemble symptoms seen in people with rheumatoid arthritis.

Other Symptoms

Dry skin and rashes
Dryness in the nose and throat
Swollen salivary glands, particularly those behind the jaw and in front of the ears
Persistent cough
Vaginal dryness

What Causes Sjögren's Syndrome?

The exact cause of Sjögren's Syndrome remains unknown. It is believed to result from a combination of genetic, environmental, and immune-system factors. Certain infections may also act as triggers in people who are genetically susceptible.

Having a family history of autoimmune diseases may increase the likelihood of developing Sjögren's Syndrome. The condition predominantly affects women.

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

Consult a healthcare professional if persistent dryness of the eyes or mouth, unexplained fatigue, joint pain, or swollen salivary glands interfere with daily activities or do not improve with routine measures.

Persistent or worsening dry eyes or dry mouth
Difficulty swallowing or speaking because of severe mouth dryness
Recurrent dental cavities or oral infections
Persistent joint pain or swelling
Unexplained fatigue that affects daily activities
Persistent swelling of the salivary glands
New or persistent symptoms affecting the lungs, kidneys, nerves, or other organs
Unexplained weight loss, persistent fever, night sweats, or enlarged lymph nodes, which require prompt medical evaluation

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How Is Sjögren's Syndrome Diagnosed?

Diagnosing Sjögren's Syndrome can be complex because its symptoms may overlap with other autoimmune and non-autoimmune conditions. A healthcare provider uses the medical history, physical examination, blood tests, eye tests, and salivary gland evaluation to support the diagnosis.

Medical History and Physical Examination

The doctor reviews symptoms, their severity, and duration and examines areas such as the eyes, mouth, salivary glands, joints, and skin.

Blood Tests

Blood tests can help identify antibodies commonly associated with Sjögren's Syndrome, particularly anti-SSA (Ro) antibodies. Other tests may evaluate inflammation, blood counts, kidney function, and other conditions that can cause similar symptoms.

Schirmer's Test

This test measures tear production to assess eye dryness. A small strip of filter paper is placed under the lower eyelid, and the amount of moisture produced is measured.

Salivary Gland Function Tests

Sialometry measures saliva flow. In selected cases, a minor salivary gland biopsy may be performed to look for characteristic inflammatory changes that support the diagnosis.

Imaging Studies

Salivary gland ultrasound may help assess the structure of the salivary glands. Other imaging tests may be used when there is concern about involvement of other organs.

How Is Sjögren's Syndrome Different From Lupus?

Sjögren's Syndrome and lupus are both autoimmune diseases and can share symptoms such as fatigue and joint pain. However, Sjögren's Syndrome is particularly associated with persistent dry eyes and dry mouth. Lupus may cause additional features such as certain skin rashes, kidney inflammation, and neurological or blood-related problems. A detailed medical evaluation and appropriate testing are needed to distinguish between these conditions.

What are the Treatment Options for Sjögren's Syndrome?

There is currently no cure for Sjögren's Syndrome, so treatment focuses on relieving dryness and other symptoms, preventing complications, and treating organ involvement when present. Treatment is individualized and may involve rheumatologists, ophthalmologists, dentists, and other specialists.

Medications

Artificial Tears and Saliva Substitutes: These products can help relieve dryness of the eyes and mouth.
Anti-inflammatory Drugs: Nonsteroidal anti-inflammatory drugs (NSAIDs) may help relieve joint pain and inflammation. Corticosteroids may be used in selected cases of significant systemic inflammation.
Immunomodulatory Drugs: Medications such as hydroxychloroquine or other immunomodulatory treatments may be considered for certain systemic symptoms under specialist supervision.
Biologic Agents: In selected patients with significant systemic disease, biologic medicines such as rituximab may be considered by a specialist.

Lifestyle and Home Care

Hydration: Drinking water regularly can help relieve dry mouth and maintain hydration.
Humidifiers: Using a humidifier can add moisture to the air and may reduce dryness of the eyes, nose, and throat.
Good Oral Hygiene: Regular dental check-ups, brushing, flossing, and appropriate fluoride use are important for reducing the risk of cavities and oral infections.
Avoiding Irritants: Limiting exposure to smoke, wind, and very dry environments can help reduce irritation and dryness.

Surgical Interventions

In selected cases of severe dry eyes, punctal plugs may be inserted into the tear ducts to reduce tear drainage and help maintain moisture on the surface of the eyes.

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What Are the Complications of Sjögren's Syndrome?

Sjögren's Syndrome can cause complications when dryness or inflammation is severe or when organs outside the glands are affected.

Dental Issues: Reduced saliva can increase the risk of cavities, oral infections, and tooth loss.
Eye Problems: Severe untreated dryness can damage the surface of the eye and may lead to corneal complications.
Lymphoma: People with Sjögren's Syndrome have an increased risk of certain types of lymphoma, although this complication is uncommon.
Organ Involvement: Inflammation can sometimes affect organs such as the lungs, kidneys, liver, or nervous system.

How Can Sjögren's Syndrome Be Managed?

Regular follow-up can help control symptoms and identify complications early. Eye examinations, dental care, adequate hydration, symptom-directed treatment, and monitoring for systemic involvement are important parts of long-term management.