Sjogren Syndrome

What is Sjogren syndrome?

Sjogren syndrome (also spelled Sjögren’s syndrome and pronounced “SHOW-grins”) is a long-term autoimmune disease. An autoimmune disease is a condition in which the body’s immune system, which normally fights infection, mistakenly attacks the body’s own healthy tissue. In Sjogren syndrome, the immune system mainly targets the glands that produce moisture, especially the tear glands in the eyes and the salivary glands in the mouth. As these glands become inflamed and damaged, they make less fluid, which leads to the two hallmark features of the condition: dry eyes and dry mouth.

Although dryness is the most recognized feature, Sjogren syndrome is a whole-body (systemic) condition. It can also affect the skin, joints, lungs, kidneys, nerves, digestive system and blood cells, and it can cause deep tiredness. Doctors often describe two forms:

• Primary Sjogren syndrome occurs on its own, without another connected autoimmune disease.
• Secondary Sjogren syndrome occurs alongside another autoimmune condition, most commonly rheumatoid arthritis (an autoimmune disease of the joints), lupus (an autoimmune disease that can affect many organs) or scleroderma (a condition that causes hardening of the skin and connective tissue).

Sjogren syndrome is one of the more common autoimmune diseases. It affects women far more often than men, and most people are diagnosed in middle age, typically after the age of 40. However, it can develop at any age, including in younger adults and, rarely, in children. Because the early symptoms can be mild or mistaken for other problems, many people live with the condition for years before it is recognized. In hospital settings, Sjogren syndrome is usually managed by a rheumatologist, a doctor who specializes in autoimmune and joint diseases, often working together with eye specialists, dentists and other doctors as needed. At Acibadem, this care is coordinated through the Rheumatology Department.

Sjogren syndrome symptoms

Sjogren syndrome symptoms vary widely from person to person. Some people have only mild dryness, while others develop symptoms in many parts of the body. Symptoms may develop slowly over months or years and can change over time. The most common symptoms include:

• Dry eyes, often described as a gritty, burning or sandy feeling, sometimes with redness, itching, blurred vision or sensitivity to light.
• Dry mouth, which can make it hard to chew, swallow dry food or speak for long periods, and may change the sense of taste.
• Dental problems, such as frequent cavities, gum disease and oral thrush (a yeast infection in the mouth), because saliva normally protects the teeth.
• Swollen salivary glands, particularly the parotid glands located in front of the ears and behind the jaw.
• Fatigue, a deep tiredness that does not improve with rest and is one of the most disabling symptoms for many people.
• Joint pain and stiffness, and sometimes muscle aches.
• Dry skin, skin rashes and, in some people, dryness of the nose, throat or vagina.
• Persistent dry cough or a hoarse voice due to dryness of the airways.
• Numbness, tingling or burning in the hands or feet, which may indicate nerve involvement (peripheral neuropathy).
• Brain fog, meaning difficulty with concentration or memory.

In primary Sjogren syndrome, dryness and fatigue are often the main features, although organ involvement can occur. In secondary Sjogren syndrome, the symptoms of the accompanying condition, such as the joint swelling of rheumatoid arthritis or the skin and kidney problems of lupus, may be more noticeable than the dryness itself, and Sjogren syndrome may be identified later.

In a smaller number of people, the disease affects internal organs. Possible complications include inflammation of the lungs, kidney problems, liver involvement, inflammation of blood vessels (vasculitis) and low blood cell counts. People with Sjogren syndrome also have a higher-than-average risk of developing lymphoma, a cancer of the lymphatic system, although this remains uncommon. Regular follow-up allows doctors to watch for these developments.

Causes and risk factors

The exact sjogren syndrome causes are not fully understood. Researchers believe the disease results from a combination of factors rather than a single trigger. In simple terms, something prompts the immune system to send white blood cells (lymphocytes) into the moisture-producing glands, where they cause inflammation and gradually reduce gland function. The immune system also produces autoantibodies, which are proteins that mistakenly target the body’s own tissues.

Factors thought to contribute include:

• Genetics. Certain inherited genes appear to make some people more likely to develop autoimmune conditions. Sjogren syndrome sometimes runs in families, although most relatives of affected people never develop it.
• Environmental triggers. A previous infection, particularly with certain viruses, may set off the abnormal immune response in people who are already genetically susceptible. No single infection has been proven to cause the disease.
• Hormones. Because the condition is much more common in women and often begins around the time of menopause, hormonal factors such as changes in estrogen are believed to play a role.
• Other autoimmune diseases. Having rheumatoid arthritis, lupus, scleroderma or autoimmune thyroid disease increases the likelihood of also developing Sjogren syndrome.

Recognized risk factors include being female, being over the age of 40, having a personal or family history of autoimmune disease, and, in some studies, certain genetic markers. Sjogren syndrome is not contagious, and it is not caused by anything a person did or failed to do.

Sjogren syndrome diagnosis

Sjogren syndrome diagnosis can be challenging because dryness, fatigue and joint pain are common and have many possible causes, including side effects of medicines, aging, anxiety and other medical conditions. There is no single test that confirms the disease. Instead, doctors combine your medical history, a physical examination and several specialized tests, and they often apply internationally agreed classification criteria that weigh the results together.

Tests your doctor may use include:

• Blood tests. Doctors commonly look for autoantibodies called anti-SSA (anti-Ro) and anti-SSB (anti-La), as well as antinuclear antibodies (ANA) and rheumatoid factor. Blood tests may also check for signs of inflammation, anemia or low white blood cell counts, and assess kidney and liver function. Not everyone with Sjogren syndrome has positive antibody results, so a negative test does not rule it out.
• Eye tests. The Schirmer test measures tear production by placing a small strip of filter paper under the lower eyelid for a few minutes. An eye doctor may also apply special dyes to the eye surface and use a slit lamp (a microscope with a bright light) to look for dry spots or damage to the cornea.
• Saliva tests. Salivary flow can be measured by collecting saliva over a set period. A salivary gland scan (scintigraphy) or ultrasound of the salivary glands may be used to look at gland structure and function.
• Lip biopsy. In a minor salivary gland biopsy, a doctor removes a few tiny glands from the inside of the lower lip under local anesthetic and examines them under a microscope for clusters of inflammatory cells. This is one of the most specific tests for Sjogren syndrome.
• Imaging and other tests. Depending on symptoms, a chest X-ray, CT scan, lung function tests, nerve conduction studies or urine tests may be ordered to check whether organs beyond the glands are involved.

Your doctor will also review your current medicines, since many common drugs, including some antidepressants, antihistamines and blood pressure medicines, can cause dryness that mimics Sjogren syndrome. Because several specialists may be involved, diagnosis is often coordinated by a rheumatologist.

Sjogren syndrome treatment options

There is currently no cure for Sjogren syndrome, but a wide range of treatments can relieve symptoms, protect the eyes and teeth, and manage complications. Treatment is individualized: someone with mild dryness may need only simple measures, while someone with organ involvement may need stronger medicines. Sjogren syndrome treatment options generally fall into the following groups.

Observation and self-care measures

For many people, the first step is regular monitoring combined with practical steps to manage dryness. These often include using artificial tears frequently, sipping water throughout the day, chewing sugar-free gum or sucking sugar-free lozenges to stimulate saliva, using a humidifier at home, avoiding smoke and drafts, and applying moisturizers to dry skin. Careful dental hygiene, including brushing with fluoride toothpaste and regular dental check-ups, is important because reduced saliva increases the risk of cavities.

Medications for dryness

• Lubricating eye drops, gels and ointments are used for dry eyes; preservative-free products are often recommended for frequent use.
• Prescription eye drops such as cyclosporine or lifitegrast may be prescribed to reduce inflammation of the eye surface and improve tear production over time.
• Saliva substitutes and moisturizing mouth sprays or gels can ease dry mouth.
• Saliva-stimulating medicines, such as pilocarpine or cevimeline, may be prescribed to increase saliva and sometimes tear production. They can cause side effects such as sweating and are not suitable for everyone.
• Antifungal treatment may be needed for oral thrush.

Medications for systemic symptoms

When Sjogren syndrome causes joint pain, fatigue, rashes or organ inflammation, doctors may prescribe medicines that act on the immune system. Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen are often used for mild joint pain. Hydroxychloroquine, a medicine originally developed for malaria and widely used in lupus, is commonly prescribed for joint pain, rashes and fatigue. Corticosteroids (steroid medicines that reduce inflammation) and immunosuppressants such as methotrexate, azathioprine or mycophenolate may be considered for more serious organ involvement. In selected cases, biologic medicines that target specific immune cells may be used. All of these medicines require monitoring for side effects, and your doctor will weigh benefits against risks.

Procedures

For persistent dry eyes, an eye doctor may suggest punctal plugs, which are tiny plugs inserted into the tear drainage ducts to keep tears on the eye surface longer. This is a quick outpatient procedure. In some cases, the ducts can be closed more permanently with a minor procedure. Surgery is rarely needed for Sjogren syndrome itself, but it may be considered for complications such as a persistently swollen or infected salivary gland, or for severe eye surface damage.

Rehabilitation and supportive care

Fatigue and joint pain can be improved for some people through paced physical activity, sleep management and, where appropriate, physical therapy. Speech and swallowing therapists can help with swallowing difficulties related to dry mouth. Because living with a chronic illness can affect mood, psychological support or counseling is a recognized part of comprehensive care.

Living with Sjogren syndrome and outlook

Sjogren syndrome is a lifelong condition, but for most people it is not life-threatening, and many are able to keep working, raising families and staying active with appropriate management. Symptoms tend to fluctuate, with better and worse periods, and dryness often gradually increases over the years. Daily attention to eye and mouth care can prevent many of the most troublesome complications, such as corneal damage and tooth loss.

The long-term outlook depends largely on whether organs beyond the glands are involved. People with dryness alone generally have a life expectancy similar to the general population. Those with lung, kidney, nerve or blood vessel involvement need closer follow-up. Because of the increased risk of lymphoma, doctors typically watch for warning signs such as persistently enlarged glands or lymph nodes, unexplained weight loss or night sweats. Most people with Sjogren syndrome never develop lymphoma, but ongoing review allows any problem to be found early.

Practical tips that many people find helpful include carrying water and eye drops at all times, protecting the eyes with wraparound sunglasses outdoors, avoiding alcohol-based mouthwashes, seeing a dentist regularly, telling every doctor and pharmacist about the diagnosis before starting new medicines, and connecting with patient support groups. Women planning pregnancy should discuss it with their doctor, because anti-SSA antibodies can, in a small number of cases, affect the baby’s heart and require extra monitoring during pregnancy.

Frequently asked questions

What is Sjogren syndrome in simple terms?

Sjogren syndrome is an autoimmune disease in which the immune system attacks the glands that make tears and saliva, causing dry eyes and dry mouth. It can also cause fatigue, joint pain and, in some people, problems in organs such as the lungs, kidneys and nerves. It is a chronic condition that is managed rather than cured.

What are the first sjogren syndrome symptoms people notice?

The earliest symptoms are often persistent dry, gritty eyes and a dry mouth that makes swallowing dry food difficult. Some people first notice unexplained tiredness, aching joints, frequent dental cavities or swelling in front of the ears. Because these symptoms are common and nonspecific, they are frequently attributed to other causes at first, which can delay recognition of the condition.

What are the main sjogren syndrome causes?

The cause is not fully known. Doctors believe that a combination of inherited genes, hormonal influences and an environmental trigger such as a viral infection leads the immune system to attack the moisture-producing glands. It is more common in women and in people who already have another autoimmune disease. It is not contagious and is not caused by lifestyle choices.

How is sjogren syndrome diagnosis confirmed?

There is no single confirming test. Doctors combine a review of symptoms with blood tests for autoantibodies such as anti-SSA and anti-SSB, eye tests that measure tear production and check the eye surface, measurements of saliva flow or salivary gland imaging, and sometimes a small biopsy from the inside of the lip. The results are usually assessed against international classification criteria by a rheumatologist.

What are the sjogren syndrome treatment options if it cannot be cured?

Treatment aims to relieve symptoms and prevent complications. Options include artificial tears and prescription eye drops, saliva substitutes, medicines that stimulate saliva, careful dental care, punctal plugs for dry eyes, and, for joint pain or organ involvement, medicines such as hydroxychloroquine, corticosteroids or immunosuppressants. Your doctor will tailor treatment to your specific symptoms and monitor for side effects.

Is Sjogren syndrome serious or life-threatening?

For most people, Sjogren syndrome is a manageable long-term condition with a life expectancy similar to the general population. It can be serious when it affects internal organs or when complications such as lymphoma develop, but these outcomes are uncommon. Regular follow-up with your care team helps detect any changes early.

Can Sjogren syndrome go away on its own?

Sjogren syndrome does not typically go away, because the underlying immune process is long-term. Symptoms can vary in intensity, and some people have long periods with few problems, but the condition generally requires ongoing management. Consistent eye and mouth care and appropriate medication can help many people keep symptoms under good control.

When to see a doctor

It is reasonable to arrange a medical review if you have had dry eyes or dry mouth for more than a few months, especially if you also have fatigue, joint pain, repeated dental cavities or swollen glands near the jaw. If you have already been diagnosed with Sjogren syndrome, contact your care team about any new or worsening symptoms. Seek urgent medical attention if you notice any of the following red-flag signs:

• Sudden eye pain, marked redness or a rapid decrease in vision, which may indicate damage or infection of the cornea.
• A salivary gland that becomes suddenly painful, hot, hard or very swollen, or a swelling that keeps growing over weeks.
• Persistently enlarged lymph nodes in the neck, armpits or groin, unexplained weight loss, fevers or drenching night sweats.
• Shortness of breath, chest pain or a new persistent cough, which may signal lung involvement.
• Blood in the urine, swelling of the legs or a sharp reduction in how much you urinate, which may point to kidney problems.
• New numbness, weakness, severe tingling or difficulty walking, which may indicate nerve involvement.
• Unusual bruising, purple spots on the skin or bleeding that does not stop easily.
• Signs of a serious infection while taking immunosuppressive medicines, such as high fever, chills or confusion.

These signs do not necessarily mean a serious complication has occurred, but they should be assessed promptly by a doctor so that the right tests and treatment can be started without delay.