Sjögren’s disease, previously called Sjögren’s syndrome, is an autoimmune disease that can look and progress very differently from person to person. Some individuals with Sjögren’s experience mild, stable symptoms for years, while others notice worsening symptoms and complications throughout their body over time. Sjögren’s does not follow a single, predictable course.

Although Sjögren’s disease does not have official or universally accepted stages, some doctors describe its progression using a stage 0-to-3 framework. This approach helps explain how symptoms and organ involvement can change over time, even though most people never progress to severe disease.

This article provides an overview of how Sjögren’s disease may progress, what doctors sometimes mean when they talk about “stages,” and how treatment changes depending on organ involvement.

What Is Sjögren’s Disease?

Sjögren’s is a long-term condition where the body’s immune system attacks its own moisture-producing glands.

The most common symptoms of Sjögren’s are:

• Dry mouth, when the saliva-producing glands are affected
• Dry eyes, when the lacrimal glands (tear-producing glands) are affected

These symptoms can be mild or cause major discomfort and complications over time.

Other symptoms of Sjögren’s disease include:

• Vaginal dryness, causing pain with sexual intercourse
• Dry or itchy skin
• Skin lesions
• Fatigue
• Joint pain
• Anemia
• Low white blood cell count
• Burning, tingling, or numbness in the arms or legs

While this condition can’t yet be cured, its symptoms can be well managed. Most people with Sjögren’s work with a rheumatologist, a doctor who specializes in autoimmune diseases, to treat their condition and monitor their symptoms over time.

The Stages of Sjögren’s Disease

Sjögren’s disease doesn’t have officially defined or numbered stages. That being said, doctors sometimes talk about Sjögren’s in informal stages (stage 0 to stage 3) to explain how symptoms and organ involvement change over time. These “stages” are best thought of as common patterns rather than fixed categories. A person may remain in one stage for life, skip stages, or experience features from more than one stage at a time.

Typically, people with Sjögren’s start with early symptoms of dry eyes and dry mouth. Some may never have any further symptoms, whereas others may start to notice systemic manifestations (full-body effects) like fatigue and joint pain. People with systemic involvement may begin to experience damage to the lungs, kidneys, or nerves.

It’s important to note that these stages are different from “primary Sjögren’s” and “associated Sjögren’s.” Primary Sjögren’s is when someone only has Sjögren’s disease. Associated Sjögren’s, formerly called secondary Sjögren’s, is when Sjögren’s appears with another autoimmune disease like rheumatoid arthritis or systemic lupus erythematosus.

Stage 0: Early or Preclinical Sjögren’s

Stage 0 is sometimes used to describe people with early immune system changes related to Sjögren’s, but with few to no symptoms. It may take years to get a diagnosis of preclinical Sjögren’s.

At this stage:

• Blood tests may show anti-Sjögren’s-syndrome-related antigen A (anti-SSA) and anti-SSB antibodies.
• The function of salivary glands or tear glands may be mildly worse.
• Dryness symptoms may be absent or very subtle.

Some people are diagnosed in this stage because of abnormal lab test results done for another reason. They may also be diagnosed if they have a different autoimmune disease. Some people may never progress beyond this point.

Treatment at this stage often focuses on:

• Monitoring any new symptoms
• Regular follow-up with a rheumatologist
• Managing any other autoimmune conditions

Stage 1: Gland Symptoms (Dry Eyes and Dry Mouth)

This stage often refers to early symptomatic Sjögren’s disease, where dryness becomes more noticeable and frequent due to involvement of the tear and saliva glands.

Common features of Sjögren’s at this stage include:

• Burning, irritation, or grittiness of the eyes
• Difficulty swallowing, speaking, or tolerating dry foods
• Dental cavities or mouth infections

Symptoms at this stage may come and go, and many people feel otherwise healthy. For many people with Sjögren’s, dryness remains the primary issue for years or decades.

Treatment and lifestyle changes at this stage often include:

• Artificial tears
• Eye lubricants
• Saliva substitutes
• Indoor humidifiers
• Medications to increase saliva production
• Moisturizer for dry skin
• Good dental and eye care to prevent complications
• A plan to quit smoking, if needed

Stage 2: Systemic Symptoms Beyond Dryness

In stage 2, Sjögren’s disease may begin to affect the body more broadly. This is sometimes called systemic Sjögren’s, and these symptoms are called “extraglandular manifestations” — when Sjögren’s affects body parts outside of the glands.

In addition to dryness, many people at this stage may experience general symptoms of an autoimmune disorder. These include:

• Persistent fatigue
• Joint pain or stiffness
• Muscle aches
• Constant cough
• Trouble breathing
• Brain fog
• Difficulty concentrating
• Mild nerve symptoms, like tingling

Although Sjögren’s inflammation is affecting more parts of the body, it is not causing organ damage. The symptoms in stage 2 can significantly impact quality of life.

Treatment at this stage may involve:

• Medications and lifestyle changes to manage dryness
• Treatment for fatigue and nervous system symptoms such as tingling
• Nonsteroidal anti-inflammatory drugs (NSAIDs) or oral corticosteroids for joint pain and stiffness
• Drugs to target the immune system

Your rheumatologist can help you decide on the immunosuppressive drug that works best for your symptoms.

Stage 3: Organ Involvement and Complications

Stage 3 is sometimes used to describe more severe Sjögren’s disease, when inflammation damages internal organs. It’s important to emphasize that most people with Sjögren’s never reach this stage.

Possible organ involvement may include:

• Lungs, causing cough or shortness of breath
• Kidneys, affecting electrolyte balance or kidney function
• Nervous system, leading to numbness, weakness, or pain
• Blood vessels, causing vasculitis (inflammation)
• Lymph nodes, causing lymphoma, a type of cancer

People with more advanced disease often require closer monitoring and more intensive treatment. Treatment options at this stage may include all or some of the following:

• Immunosuppressive medication
• Organ-specific treatments
• Biologic drugs

At this stage, people with Sjögren’s should regularly follow up with their autoimmune doctor and other specialists to monitor disease activity and adjust their treatments over time.

How Quickly Does Sjögren’s Progress?

There is no single timeline for Sjögren’s disease progression. Some people remain stable for many years, while others notice gradual changes. Factors that may influence progression include:

• Having an older age at diagnosis
• Being female
• Having other autoimmune diseases
• Getting an early diagnosis
• Starting treatment quickly

If you’re living with Sjögren’s, it’s important to follow up with your doctor regularly to catch complications early. Keep track of any new or worsening symptoms with a symptom journal and keep your medical team updated.

Living With Sjögren’s Over Time

Sjögren’s can be unpredictable, but many people live full and active lives with appropriate care. Understanding common patterns of progression can help you know what symptoms to watch for and what questions to ask your doctor.

Staying engaged in your care, attending regular appointments, and communicating openly with your healthcare team are key parts of managing Sjögren’s at any stage.

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