INTRODUCTION TO DIAGNOSIS
Sjogren’s is a clinical diagnosis that is supported by, but cannot be excluded by, diagnostic testing.
Sjogren’s is a clinical diagnosis
A clinical diagnosis takes into account the big picture. This includes a detailed medical history, family history, physical examination, and laboratory tests. Patients typically seek diagnosis when symptoms are sudden, severe, or obviously abnormal. Many Sjogren’s manifestations tend to come on slowly. Patients may not mention them, thinking they are unimportant or a normal part of aging. Clinicians must learn common presentations and the specific questions that could lead them to suspect Sjogren’s.
While laboratory results may support diagnosis, normal test results do not rule out Sjogren’s. Some rheumatologists insist that Classification Criteria be met for diagnosis. This often results in long delays or misdiagnosis, especially in patients who are seronegative for Sjogren’s antibodies. The most recent Classification Criteria (2016) rely solely on objective measures, which do not adequately reflect the systemic nature of the disease. They were created as a tool to standardize research populations, which is a good thing. However, due to fundamental limitations, these criteria do not substitute for clinical judgment by an experienced rheumatologist. In his excellent review article, Sjogren's expert Dr. Fredrick Vivino explains the problem with requiring either positive anti-SSA or minor salivary gland biopsy for a Sjogren's diagnosis. “Until we have better tests, 'the gold standard' for diagnosis still remains the clinician’s expert opinion." (2)
Why is timely diagnosis important?
Diagnosis is critical for access to appropriate clinical management. Treatment improves quality of life and can reduce progression and prevent complications.
A “Sjogren’s knowledgeable” health care team should be assembled as early as possible.At a minimum, this includes a primary care provider, rheumatologist, ophthalmologist, dentist, and for women, a gynecologist. Other specialists may be indicated.
It is important to look for autoimmune diseases that often occur together with Sjogren’s. Some of the more common ones include thyroid disease, celiac disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE).
Sjogren’s patients should be routinely monitored for direct Sjogren’s complications such as organ system involvement, neurologic manifestations and lymphoma.
Patients diagnosed with Sjogren’s are at increased risk for cardiovascular disease, osteoporosis and infections. PCPs play a key role in prevention and management of these conditions.
HIV and hepatitis C virus (HCV) can mimic Sjogren’s. They should be ruled out.
Why is Sjogren’s diagnosis so often delayed?
Lack of attention to Sjogren's in medical education, misperceptions, and disinterest at all levels in the health care system cause Sjogren’s to be “missed and dismissed.” Few clinicians are trained to recognize even typical Sjogren’s presentations.
Why aren’t there better tests to diagnose Sjogren’s?
This will hopefully change soon. The SSF supports researchers who are looking for biomarkers specific to Sjogren’s. These novel biomarkers could be game-changers. Being able to diagnose Sjogren’s early and accurately could make a profound difference in our understanding of the disease.
The diagnosis section pertains to adult patients.
This website addresses Sjogren’s in adult patients. While Sjogren’s is not common in children, it is seen. Presentations are often incomplete, and diagnosis can be tricky. Children with Sjogren’s often present with atypical clinical features. This website does not attempt to address pediatric Sjogren's.