From the Guidelines
Sjögren's syndrome is diagnosed based on a combination of clinical symptoms, laboratory tests, and histopathological findings, particularly the presence of anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2 1.
Key Diagnostic Criteria
- Dry eye and dry mouth associated with systemic immune dysfunction 1
- Anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2, each scoring 3 1
- Abnormal ocular staining score of ≥5, Schirmer test result of ≤5 mm/5 minutes, and unstimulated salivary flow rate of ≤0.1 ml/minute, each scoring 1 1
- A total score of ≥4 for these items meets the criteria for primary Sjögren's syndrome 1
Histopathological Findings
- Labial salivary gland biopsy is widely used in the diagnosis of primary Sjögren's syndrome and plays an integral role in the established American-European Consensus Group classification criteria and the proposed American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria 1
- Focus score is calculated by counting the number of foci, whose area is delineated within the black lines, dividing by the whole glandular surface area in mm2 and multiplying by 4 to give the number of foci per 4 mm2 over the whole glandular area 1
Clinical Considerations
- Sjögren's syndrome should be suspected if intrinsic tear-production deficiency is detected in nonelderly women, especially if it is rapid in onset and/or marked in severity 1
- Diagnosis and treatment of underlying systemic immune disorders may decrease morbidity and may even be lifesaving 1
- Patients with Sjögren's syndrome are at increased risk for potentially life-threatening vasculitic or lymphoproliferative disorders 1
From the Research
Diagnosis of Sjögren's Syndrome
The diagnosis of Sjögren's syndrome is based on a combination of clinical and biological evidence, including:
- Characteristic clinical signs and symptoms, such as ocular and oral dryness resulting from lacrimal and salivary gland dysfunction 2
- Specific tests, including salivary gland histopathology and autoantibodies 2, 3
- Detection of autoantibodies in patient serum and histological analysis of biopsied salivary gland tissue 3
- Objective evidence of dry eyes and/or objective evidence of dry mouth, as well as proof of autoimmunity 4
Diagnostic Criteria
The diagnosis of Sjögren's syndrome can be made according to the American College of Rheumatology and European League Against Rheumatism (EULAR) classification criteria, which involve clinical, serological, and histological assessment 5. Recently, new classification criteria and disease activity scores have been developed, primarily for research purposes, but can also be useful tools in everyday clinical practice 2.
Challenges in Diagnosis
The diagnosis of Sjögren's syndrome is often difficult due to the heterogeneous clinical presentation of the disease, which can vary from sicca symptoms to systemic disease 3. The development of new imaging techniques can help the clinician in the diagnostic approach 6.