From the Guidelines
The diagnostic workup for Sjögren's syndrome should begin with a thorough clinical evaluation focusing on sicca symptoms (dry eyes and dry mouth), followed by specific laboratory and diagnostic tests, as outlined in the most recent guidelines 1. The initial evaluation should include a comprehensive assessment of the patient's symptoms, medical history, and physical examination.
- Key symptoms to evaluate include dry eyes, dry mouth, and other systemic symptoms such as arthralgia, myalgia, or fatigue.
- The patient's medical history should be reviewed for any underlying autoimmune diseases, such as rheumatoid arthritis, scleroderma, or systemic lupus erythematosus.
- A physical examination should be performed to assess for any signs of ocular or oral involvement, such as conjunctivitis, keratitis, or salivary gland enlargement.
Initial blood tests should include:
- Antinuclear antibodies (ANA)
- Anti-SSA/Ro and anti-SSB/La antibodies
- Rheumatoid factor
- Complete blood count
- Comprehensive metabolic panel
- Erythrocyte sedimentation rate
- C-reactive protein These tests can help identify any underlying autoimmune disorders and assess the patient's overall inflammatory status 1.
Ocular assessment should include:
- Schirmer's test (measuring tear production; less than 5mm wetting in 5 minutes is abnormal)
- Ocular surface staining with fluorescein or lissamine green to evaluate corneal damage These tests can help assess the severity of dry eye and identify any corneal damage 1.
Oral evaluation should include:
- Assessment of unstimulated salivary flow rate (less than 0.1 mL/minute is abnormal)
- Possibly a labial salivary gland biopsy, which remains the gold standard for diagnosis, looking for focal lymphocytic sialadenitis with a focus score ≥1 These tests can help assess the severity of dry mouth and identify any underlying salivary gland dysfunction 1.
Additional testing may include:
- Salivary gland ultrasonography
- Sialography
- Salivary scintigraphy These tests can help assess the structure and function of the salivary glands and identify any underlying abnormalities 1.
The diagnosis of Sjögren's syndrome should be based on the classification criteria, which include a combination of clinical and laboratory findings, such as anti-SSA/Ro antibody positivity, focal lymphocytic sialadenitis, and abnormal ocular staining score 1. Early diagnosis is important as it allows for appropriate management of both glandular and extraglandular manifestations of the disease, which can help reduce morbidity and improve quality of life 1.
From the Research
Diagnostic Workup for Sjögren's Syndrome
The diagnostic workup for Sjögren's syndrome involves a combination of clinical evaluation, laboratory tests, and imaging studies. The following are the key components of the diagnostic workup:
- Establishment of a dry eye diagnosis using patient questionnaires and objective ocular tests, including inflammatory biomarker testing 2
- Schirmer test and tear film break-up time to differentiate between aqueous-deficient dry eye (ADDE) and evaporative dry eye 2
- Serological tests for autoantibody biomarkers, such as SS-A/Ro, SS-B/La, antinuclear antibody, and rheumatoid factor 2, 3
- Minor salivary gland and lacrimal gland biopsies to evaluate for structural damage and secretory dysfunction 2, 4
- Examination of newer biomarkers, such as autoantibodies to salivary gland protein-1, parotid secretory protein, and carbonic anhydrase VI, which may allow for earlier diagnosis of Sjögren's syndrome 2, 3
- Use of a commercially available diagnostic test kit, such as the Sjö(®) test, which incorporates traditional and novel biomarkers 2, 3
Diagnostic Criteria
The diagnosis of Sjögren's syndrome is made based on the presence of signs and symptoms suggestive of the disease, along with at least two objective factors, such as:
- Traditional biomarker positivity (e.g. SS-A/Ro, SS-B/La, antinuclear antibody, rheumatoid factor) 3
- Salivary gland biopsy findings 3, 4
- Presence of keratoconjunctivitis sicca 3
- Novel biomarker positivity (e.g. autoantibodies to salivary gland protein-1, parotid secretory protein, carbonic anhydrase VI) 2, 3
Collaboration and Referral
Diagnosis and management of Sjögren's syndrome often require collaboration among multiple specialties, including rheumatology, ophthalmology, and oral care professions 3, 5. Referral to a rheumatologist or other specialist may be necessary for further evaluation and treatment.