Sjögren’s disease, previously called Sjögren’s syndrome, is an autoimmune disease. In autoimmune diseases, the immune system mistakenly attacks healthy parts of the body.

If you or a loved one has been diagnosed with Sjögren’s, you might hear the terms primary Sjögren’s disease and secondary Sjögren’s disease. These are two commonly used terms to classify Sjögren’s. The difference between them isn’t due to severity or symptoms — it’s whether or not you have another autoimmune disease.

Understanding the difference between primary and secondary Sjögren’s can be useful in talking with your doctor and learning more about your condition. There are also a few important details about how these terms are used. Below, you’ll learn more about the types of Sjögren’s and the treatment options linked to each one.

Types of Sjögren’s Disease

Sjögren’s disease affects the glands in your body that produce moisture and can lead to long-term dryness of the eyes, mouth, nose, throat, and other body parts. The two main forms are primary and secondary. They’re very similar, but there’s one key difference.

Primary Sjögren’s

Sjögren’s disease is generally associated with other autoimmune conditions, but it can also develop on its own. When this happens, it’s called primary Sjögren’s.

Having primary Sjögren’s doesn’t mean you won’t develop another autoimmune disorder later. If you do, your Sjögren’s diagnosis will shift to secondary Sjögren’s.

Secondary Sjögren’s

Secondary Sjögren’s is when the disease occurs with another autoimmune disease. Around half of all people with Sjögren’s also have another autoimmune condition. Sjögren’s may appear before, after, or at the same time as the other condition.

These are other autoimmune conditions that are commonly linked to Sjögren’s:

• Systemic lupus erythematosus, also called lupus — Can affect the skin, joints, and organs
• Rheumatoid arthritis — Mainly affects the joints
• Psoriatic arthritis — Joint inflammation linked to psoriasis

Viruses can trigger secondary Sjögren’s. This includes hepatitis C, Epstein-Barr virus, cytomegalovirus, HTLV-1, and COVID-19.

People with secondary Sjögren’s may be more likely to have serious complications because more conditions can affect their health.

Associated Sjögren’s Disease

Some doctors consider the term “secondary” to be misleading because it can imply that Sjögren’s disease is less of a concern compared to the other autoimmune diseases it’s associated with. It can also imply that it develops after or as a symptom of the other disease.

Sjögren’s disease can be quite severe and have a significant impact on your quality of life. Also, people who have Sjögren’s and another autoimmune disease often get Sjögren’s before the other disease develops.

As a result, many health doctors now use the preferred term associated Sjögren’s instead of secondary Sjögren’s. Healthcare providers might say, “Sjögren’s associated with lupus,” for example.

Updating the terminology could also be helpful when it comes to clinical trials. Doctors consider Sjögren’s to be generally under-researched, especially when it’s associated with other conditions. Many clinical trials that focus on Sjögren’s disease exclude people who also have another autoimmune disease. They do this because it can be difficult to know whether a drug is working in the way it’s intended to, since there are other conditions to consider. However, most people with Sjögren’s have comorbidities (additional conditions) that could affect treatment either way, and it’s important to study that relationship.

Symptoms of Primary vs. Secondary Sjögren’s Disease

When you have another autoimmune condition, you’ll likely have the symptoms of that disease in addition to Sjögren’s symptoms. People with associated Sjögren’s will likely experience a broader range of symptoms than those with primary Sjögren’s.

If you have lupus or rheumatoid arthritis, some symptoms of these conditions overlap, and it may be difficult to tell which condition is causing your symptoms, or if some symptoms may be caused by both. For example, all three conditions can cause joint pain and fatigue.

In addition to Sjögren’s symptoms, people with associated Sjögren’s disease and rheumatoid arthritis can experience weakness and fever. People with lupus and associated Sjögren’s may have symptoms like headache, fever, hair loss, and rashes.

Whether or not you have another autoimmune condition, Sjögren’s disease often causes:

• Dry eyes, mouth, and skin
• Mouth sores
• Nosebleeds
• Coughing
• Vaginal dryness
• Nerve pain
• Skin rashes and itchiness
• Swollen lymph nodes or salivary glands

Treatment for Primary vs. Secondary Sjögren’s

The goals of treatment for Sjögren’s disease are the same for primary and associated forms. In both cases, the main goals of treatment are to improve symptoms and prevent the additional problems that these symptoms can cause, like tooth decay, mouth infections, and eye damage.

Treatments for Sjögren’s often include:

• Artificial tears (eye drops) to help with dry eyes
• Surgery to close your tear ducts
• Prescriptions or supplements that help make saliva
• Corticosteroids (steroids) to reduce inflammation
• Immunosuppressive drugs to calm an overactive immune system

Although scientists haven’t found any genetic difference between primary or associated Sjögren’s, they have found that in people with associated Sjögren’s, certain treatments for the other disease may work better in people who also have Sjögren’s. For example, in people with both lupus and Sjögren’s, B-cell-targeted therapy works better than it does in people who have lupus alone.

Additionally, Sjögren’s can make the other condition worse than it is in people who don’t have Sjögren’s. For example, people with Sjögren’s and rheumatoid arthritis may have more severe arthritis and more bone damage. This can affect your treatment plan. Researchers are still studying treatments for Sjögren’s and other autoimmune diseases.

Finding the Right Plan for You

For each person, a treatment plan should look at their overall health and symptoms. This can make it harder to find the right treatment, but it can also help. For example, some treatments may be more likely to ease dry mouth in people who have both Sjögren’s and lupus.

No matter which type of Sjögren’s you have, treatment usually focuses on relieving dryness and protecting your eyes, mouth, and overall health. If you have Sjögren’s disease along with another autoimmune condition, your care plan may need to cover both. The good news is that many people find relief with the right mix of treatments and self-care. If your symptoms have changed or if you’re not getting enough relief, talk with your healthcare provider about other options. You’re not alone — support and practical advice can make a real difference.

Join the Conversation

On MySjögrensTeam, people share their experiences with Sjögren’s disease, get advice, and find support from others who understand.

What was it like getting diagnosed with Sjögren’s? Let others know in the comments below.