At what age is Sjögren's syndrome usually diagnosed?

While Sjögren's syndrome can be diagnosed at any age, the vast majority of patients receive their diagnosis after the age of 40. This autoimmune condition can be challenging to identify, leading to significant diagnostic delays, despite earlier symptom onset.

Quick Summary

Sjögren's syndrome most commonly receives a diagnosis during middle age, typically between 40 and 60 years old, with women being disproportionately affected. Symptom onset, however, can occur much earlier, even in childhood, but is often mild or misattributed, leading to diagnostic delays that can span years.

Key Points

• Peak Diagnostic Age: Sjögren's syndrome is most often diagnosed in people over 40, with peak incidence in middle age, though symptoms can begin earlier.

• Diagnostic Delay is Common: Due to non-specific symptoms and a lack of awareness, many patients experience significant delays of several years before receiving an accurate diagnosis.

• Symptoms Vary by Age: In children and younger adults, typical adult dry eye and mouth symptoms are often absent, with rashes, fatigue, and parotitis being more common indicators.

• Multi-Specialist Approach: Diagnosis typically requires a rheumatologist's coordination and involves a combination of blood tests, eye exams, and potentially biopsies.

• Gender Bias Impacts Diagnosis: The high prevalence among women, combined with a history of dismissal of female patients' symptoms, contributes to diagnostic delays.

• Early Detection is Crucial: Timely diagnosis is important for preventing complications such as corneal damage, severe dental issues, and lymphoma.

Peak Diagnostic Age: A Closer Look

Although Sjögren's syndrome can be diagnosed in individuals of all ages, including children, research confirms a concentrated period for diagnosis. A systematic literature review highlighted that the average age at diagnosis ranges from 40 to 67 years, with a particularly high incidence among women over 40. Another study focusing on primary Sjögren's syndrome found the average age of onset was 52.7 years. The onset can be insidious, with initial, milder symptoms frequently overlooked or misinterpreted.

The Problem of Delayed Diagnosis

One of the most pressing issues surrounding Sjögren's is the substantial delay between symptom onset and formal diagnosis. The Sjögren's Foundation reports a diagnostic delay that, while improving, still averaged 2.8 years in 2016 for those actively seeking a diagnosis, with some experiencing delays of over a decade. This delay can be attributed to several factors:

• Non-specific and varied symptoms: Symptoms like fatigue, joint pain, and dry eyes or mouth overlap with many other conditions, including menopause, fibromyalgia, and chronic fatigue syndrome.
• Lack of awareness: Many healthcare providers, particularly outside of rheumatology, may not be adequately trained to recognize the systemic nature of Sjögren's, often viewing it as a minor dryness issue.
• Fragmented care: The need for coordination between multiple specialists (e.g., ophthalmologists, dentists, rheumatologists) can complicate and slow the diagnostic process.
• Gender bias: The vast majority of patients are women, and their symptoms may be dismissed or attributed to psychological factors, further delaying proper investigation.

Sjögren's Syndrome in Younger Patients and Children

While less common, Sjögren's can affect children and younger adults. However, the symptoms often present differently than in older adults, and this, combined with general unawareness, increases the risk of misdiagnosis or delayed care. For example, dry eyes and mouth are less frequent in pediatric cases, with common symptoms including:

• Unexplained rashes
• Persistent fatigue and malaise
• Recurrent or persistent parotitis (swollen salivary glands)
• Arthralgia (joint pain)

These presentations often do not align with the 2016 ACR/EULAR classification criteria, which were developed primarily for adult populations, creating further diagnostic challenges. Early recognition and treatment are crucial to prevent long-term complications.

Diagnostic Tests and Criteria

Diagnosing Sjögren's is a process that relies on combining clinical symptoms, specific tests, and expert assessment. There is no single definitive test, so a rheumatologist will use a combination of approaches. Tests may include:

• Blood tests: To check for specific autoantibodies, particularly anti-SSA (Ro) and anti-SSB (La), as well as rheumatoid factor and antinuclear antibodies (ANA).
• Eye tests: Such as the Schirmer test, which measures tear production using filter paper placed under the eyelid, and ocular surface staining to examine the cornea.
• Salivary gland function tests: Including sialometry (measuring saliva flow) or imaging such as salivary scintigraphy.
• Lip biopsy: A minor procedure to examine salivary gland tissue for characteristic inflammatory cell clusters.

The 2016 ACR/EULAR classification criteria provide a framework, but as noted, they may not perfectly capture the full spectrum of the disease, particularly in children.

Comparison of Age-Related Presentations

To illustrate the diagnostic differences, consider the varied ways Sjögren's can manifest across different age groups. This table highlights typical characteristics, though individual experiences can vary widely.

Feature	Common Presentation in Middle-Aged Adults (40-60)	Common Presentation in Children/Young Adults (<35)
Primary Symptoms	Dry eyes and dry mouth (sicca symptoms) are prominent and often the main complaint.	Dryness is less common or noticeable; often present with systemic symptoms like rashes or fatigue.
Other Manifestations	Joint pain, fatigue, and swollen salivary glands are frequent. Systemic involvement (lungs, kidneys) can occur.	Recurrent or persistent parotitis (salivary gland swelling) is a key feature. Rashes and joint pain are also common.
Associated Conditions	Often overlaps with other autoimmune diseases like rheumatoid arthritis or lupus.	Can occur as part of neonatal lupus syndrome in newborns whose mothers have Sjögren's antibodies.
Diagnostic Challenges	Symptoms can be mistaken for menopause, medication side effects, or other common conditions, leading to delays.	Misdiagnosed or overlooked due to lack of awareness and atypical presentation, and often fails to meet standard criteria.

Conclusion

Sjögren's syndrome is an autoimmune disease most commonly diagnosed during middle age, particularly in women. However, the diagnostic journey is often long and complex, with initial symptoms appearing much earlier in life. Recognizing that the disease can affect different age groups in different ways is critical for a timely diagnosis. Early intervention and comprehensive care, often involving a team of specialists, can effectively manage symptoms and reduce the risk of serious long-term complications. For more information on Sjögren's, including finding support and current research, visit the Sjögren's Foundation website.

Frequently Asked Questions

Is Sjögren's syndrome only diagnosed in older people?

No, while the average diagnosis age is over 40, Sjögren's syndrome can affect people of any age, including children. The presentation can be different in younger patients, with dryness being less prominent.

What is the average age of diagnosis for Sjögren's syndrome?

The average age of diagnosis varies slightly in different studies, but is generally reported to be between 40 and 60 years old. Onset can be even earlier, sometimes in the 30s or 40s.

Do men get Sjögren's syndrome?

Yes, but it is far less common in men. Sjögren's syndrome is diagnosed in women about nine times more often than in men.

Why is Sjögren's syndrome often diagnosed late?

Diagnosis is often delayed because symptoms like fatigue and dryness can be attributed to other common conditions or menopause. The disease's systemic and varied nature means it takes time for a pattern to emerge and be recognized by a rheumatologist.

Can children have Sjögren's syndrome?

Yes, children can develop Sjögren's syndrome, but their symptoms often differ from adults. They are more likely to experience rashes, fatigue, or swollen salivary glands rather than just dryness.

What are the first signs of Sjögren's syndrome in middle age?

In middle-aged adults, the most common initial signs are persistent dry eyes and dry mouth. Some may also experience joint pain, swelling, and chronic fatigue.

Does having another autoimmune disease increase the risk of Sjögren's?

Yes. Sjögren's syndrome is classified as secondary when it occurs alongside another autoimmune disease, such as rheumatoid arthritis or lupus, which increases the likelihood of diagnosis.