Sjögren’s syndrome is a chronic autoimmune disease where the body’s own immune system mistakenly attacks the glands that produce moisture, leading to widespread dryness that can affect not only the eyes and mouth but the entire body and internal organs.

Understanding Sjögren’s Syndrome

Sjögren’s syndrome, pronounced “SHOW-grins,” is a condition that occurs when your immune system turns against your own body instead of protecting it. This autoimmune disease—meaning a disease where the immune system attacks healthy cells—primarily targets the glands responsible for producing moisture throughout your body. The most commonly affected glands are those that create tears in your eyes and saliva in your mouth, but the condition can reach far beyond these areas^[1].

The disease was first identified in the early 1900s by Swedish physician Henrik Sjögren, who noticed a pattern among women whose chronic arthritis came alongside persistent dry eyes and dry mouth. Today, medical professionals understand that this syndrome is more complex than originally thought, often affecting multiple body systems and presenting differently from one person to another^[5].

Sjögren’s syndrome can develop in two distinct ways. Primary Sjögren’s syndrome occurs on its own, without any other underlying condition causing it. Secondary Sjögren’s syndrome develops when another health issue, particularly another autoimmune disease, triggers the condition. About half of all cases occur alongside other autoimmune disorders such as rheumatoid arthritis, lupus, or scleroderma^[2].

Epidemiology: Who Gets Sjögren’s Syndrome?

Sjögren’s syndrome affects approximately two million people in the United States, with estimates ranging from one to four million depending on the source. The condition can strike anyone regardless of race or ethnicity, but certain groups face significantly higher risk than others^[3][7].

Women bear the overwhelming burden of this disease. More than 90 percent of people diagnosed with Sjögren’s syndrome are women, creating a female-to-male ratio of approximately 10 to 1. While men can and do develop the condition, it remains far less common in the male population. This dramatic gender difference suggests that hormonal factors may play an important role in the disease’s development^[4][13].

Age also matters considerably when it comes to Sjögren’s syndrome. Although the condition can develop at any age, including in children, most people receive their diagnosis between the ages of 45 and 55. Some sources indicate that the majority of patients are older than 40 at the time of diagnosis, with a second peak occurring after menopause when estrogen levels naturally decline and dryness becomes more pronounced throughout the body^[1][4][10].

People who already have other autoimmune diseases face elevated risk of developing Sjögren’s syndrome. Around half of all people with Sjögren’s syndrome have at least one other autoimmune condition. This connection is so strong that healthcare providers specifically monitor patients with rheumatoid arthritis, lupus, and similar diseases for signs of developing Sjögren’s syndrome^[3].

Causes of Sjögren’s Syndrome

The exact cause of Sjögren’s syndrome remains a mystery that scientists continue to investigate. What is known is that the disease represents a malfunction of the immune system, where the body’s defense mechanism begins attacking its own moisture-producing glands instead of protecting them from genuine threats^[3].

Primary Sjögren’s syndrome appears without any identifiable trigger or preceding condition. Researchers believe that several factors likely work together to create the perfect storm for disease development. Genetic factors appear to play a significant role—several genes have been identified that may make certain individuals more susceptible to developing the syndrome. However, having these genes does not guarantee that someone will develop the disease^[7].

Environmental factors may also contribute to triggering the disease in genetically vulnerable individuals. Some scientists theorize that Sjögren’s syndrome might be sparked by a previous infection with a virus or bacteria. Once activated, the immune system becomes confused and continues attacking the body’s own tissues long after any infection has cleared^[7].

Secondary Sjögren’s syndrome has clearer triggers, developing in response to other existing health conditions. Certain viral infections have been linked to triggering the syndrome, including hepatitis C, cytomegalovirus, Epstein-Barr virus, Human T-lymphotropic virus 1, and even COVID-19. Any autoimmune disease can potentially trigger secondary Sjögren’s syndrome, with rheumatoid arthritis, psoriatic arthritis, and lupus being particularly common culprits^[3].

Risk Factors

Several factors increase the likelihood of developing Sjögren’s syndrome, though having risk factors does not mean someone will definitely develop the disease. Understanding these risk factors helps individuals and healthcare providers remain vigilant for early signs of the condition.

Being female represents the single strongest risk factor for Sjögren’s syndrome. Women are nine to ten times more likely than men to develop the condition. This dramatic difference suggests that sex hormones, particularly estrogen, may influence the disease’s development. The two age peaks—one during childbearing years between 20 and 30, and another after menopause—further support the connection between hormonal changes and disease onset^[4][10].

Age plays a significant role in risk assessment. While Sjögren’s syndrome can develop at any age, including in children, the risk increases substantially for people between 45 and 55 years old. However, a growing number of patients are being diagnosed under age 18, indicating that the disease does not exclusively target older adults^[2].

Having another autoimmune disease dramatically increases the risk of developing Sjögren’s syndrome. Approximately half of all people with Sjögren’s syndrome have at least one other autoimmune condition. The presence of rheumatoid arthritis, lupus, scleroderma, or similar diseases should prompt both patients and doctors to watch carefully for signs of developing dryness and other symptoms characteristic of Sjögren’s syndrome^[3].

Previous viral infections may also elevate risk, though the exact mechanisms remain under investigation. Infections with hepatitis C, cytomegalovirus, Epstein-Barr virus, Human T-lymphotropic virus 1, or COVID-19 have all been associated with triggering secondary Sjögren’s syndrome in some individuals^[3].

Symptoms of Sjögren’s Syndrome

The symptoms of Sjögren’s syndrome vary dramatically from person to person. Some individuals experience only mild discomfort that barely interrupts their daily routine, while others suffer debilitating symptoms that profoundly impact their quality of life and ability to function. The condition affects people differently, and symptoms can fluctuate over time, with periods when they worsen—called flare-ups—alternating with times when they improve, known as remission^[6].

The two hallmark symptoms that define Sjögren’s syndrome are dry eyes and dry mouth. These occur because the immune system attacks and damages the lacrimal glands (which produce tears) and the salivary glands (which produce saliva). People with dry eyes often describe a burning, itching, or gritty sensation, as if sand or gravel is trapped in their eyes. The eyes may appear red and feel irritated. Dry mouth creates a feeling as though the mouth is stuffed with cotton, making swallowing and speaking difficult. The tongue may feel dry and cracked, and some people notice an unpleasant taste in their mouth caused by stomach acid reflux^[1][4].

Beyond the eyes and mouth, dryness can affect many other parts of the body. The skin may become dry and itchy. The nose can feel dry, leading to frequent nosebleeds. The throat may feel parched, triggering a persistent dry cough. Women commonly experience vaginal dryness, which can cause discomfort during intercourse. These widespread moisture problems occur because Sjögren’s syndrome can attack any of the body’s moisture-secreting glands^[3][6].

Chronic dryness brings its own set of complications. A persistently dry mouth dramatically increases the risk of tooth decay, cavities, tooth loss, gum disease, and oral infections including thrush. The lack of saliva makes chewing and swallowing difficult, potentially leading to weight loss and malnutrition. Dry eyes increase vulnerability to eye infections, corneal damage, and vision problems. Some people become unusually sensitive to light^[4][22].

Many people with Sjögren’s syndrome experience symptoms beyond dryness. Joint pain, swelling, and stiffness are common, as are muscle pain and weakness. The salivary glands may become visibly swollen, particularly those located behind the jaw and in front of the ears. Profound fatigue—a bone-deep exhaustion that doesn’t improve with rest—affects many patients and can be one of the most challenging symptoms to manage. Some people develop brain fog, experiencing difficulty thinking clearly or focusing on tasks. Skin rashes may appear, and some individuals lose their sense of taste^[1][3].

Up to half of all people with Sjögren’s syndrome develop extraglandular involvement, meaning the disease affects organs and tissues beyond the moisture-producing glands. The lungs, gastrointestinal tract, kidneys, liver, pancreas, blood vessels, and nervous system can all be impacted. This may cause symptoms such as shortness of breath due to lung inflammation and scarring, digestive problems including heartburn, numbness or tingling in the hands or feet due to neuropathy (nerve damage), and swollen lymph nodes. Rarely, people with Sjögren’s syndrome face an increased risk of developing lymphoma, a type of cancer affecting the lymphatic system^[2][4][5].

Prevention

Currently, there is no known way to prevent Sjögren’s syndrome from developing. Because the exact causes remain unclear and likely involve a combination of genetic predisposition and environmental triggers, specific prevention strategies have not been identified. However, individuals with risk factors—particularly those with other autoimmune diseases—can benefit from awareness and early detection.

For people who already have Sjögren’s syndrome, preventing complications and managing symptoms becomes the focus. Regular dental check-ups every six months are crucial for preventing tooth decay and gum disease that can result from chronic dry mouth. Brushing teeth two or three times daily with fluoride toothpaste and flossing regularly helps protect oral health^[4][6].

Regular eye examinations with an ophthalmologist help detect and prevent corneal damage and eye infections before they become serious. Protecting the eyes from environmental factors that worsen dryness—such as wind, dust, and dry air—can reduce symptoms and complications. Wearing sunglasses with enclosed sides when outdoors helps shield the eyes from drying elements^[4][6].

Maintaining overall health through a balanced diet, adequate hydration, sufficient sleep, and regular exercise may help reduce the frequency and severity of flare-ups. Avoiding smoking is particularly important, as tobacco use can irritate the eyes and mouth while causing saliva to dry up faster. Identifying and managing stress, which can trigger or worsen symptoms, also plays a valuable role in managing the disease^[6][16].

Pathophysiology: How Sjögren’s Syndrome Affects the Body

Understanding what happens inside the body during Sjögren’s syndrome helps explain why symptoms develop and progress the way they do. At its core, the disease involves a malfunction of the immune system, specifically the cells that normally defend the body against infections and other threats.

In Sjögren’s syndrome, certain white blood cells called lymphocytes become confused and begin attacking the body’s own moisture-producing glands. These lymphocytes infiltrate the lacrimal glands (which make tears) and salivary glands (which make saliva), causing inflammation and damage. Over time, this ongoing attack leads to scarring of the gland tissue, which progressively reduces the glands’ ability to produce moisture. Eventually, tear and saliva production can decline dramatically or even cease altogether^[1][5].

The inflammation and scarring process doesn’t stop with the tear and salivary glands. The same immune malfunction can affect other exocrine glands throughout the body—glands that secrete substances through ducts. This explains why people with Sjögren’s syndrome experience dryness in multiple locations: the nose, throat, skin, digestive system, and vagina all contain glands that can be targeted by the misdirected immune response^[3].

In approximately half of patients, the disease extends beyond the glands to affect internal organs and body systems. The immune system may attack the joints, causing inflammation that leads to pain, swelling, and stiffness similar to arthritis. The lungs can develop inflammation and scarring, which impairs breathing. The kidneys may be affected, altering their ability to filter waste from the blood. The nervous system can be damaged, resulting in neuropathy with numbness, tingling, or pain in the hands and feet. The gastrointestinal system may malfunction, causing digestive problems^[2][5].

The chronic inflammation throughout the body contributes to systemic symptoms like profound fatigue, muscle pain, and brain fog. The immune system remains constantly activated, essentially keeping the body in a continuous state of alert that exhausts physical and mental resources. This helps explain why rest alone often fails to relieve the deep fatigue that many patients experience^[2].

The immune system’s attack on the salivary glands creates a cascade of problems beyond simple dryness. Saliva plays numerous protective roles in the mouth: it helps wash away food particles and bacteria, neutralizes acids produced by bacteria, provides disease-fighting substances, and enhances the ability to taste and digest food. Without adequate saliva, the mouth becomes vulnerable to tooth decay, gum disease, oral infections, and difficulty eating and speaking. The loss of these protective functions explains why dental problems become so common and severe in people with Sjögren’s syndrome^[22].

Similarly, tears do more than simply moisturize the eyes. They provide essential nutrients to the cornea, wash away irritants and debris, and contain antibodies that fight infection. When tear production diminishes, the eyes become vulnerable to infections, the cornea can be damaged, and vision problems may develop. The gritty, painful sensation many patients describe results from the eye’s surface becoming dry and irritated without the protective tear film^[22].