What are the diagnostic criteria for Sjögren's (Sjögren's syndrome)?

From the Guidelines

Sjögren's syndrome should be diagnosed based on a combination of clinical symptoms, physical examination findings, and laboratory tests, with the latest classification criteria requiring a total score of ≥4 based on five items: anti-SSA/Ro antibody positivity, focal lymphocytic sialadenitis, abnormal ocular staining score, Schirmer test result, and unstimulated salivary flow rate. The diagnosis of Sjögren's syndrome is complex and requires a comprehensive approach, considering both the clinical presentation and laboratory findings 1.

Key Diagnostic Criteria

• Anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2, each scoring 3
• Abnormal ocular staining score of ≥5 (or van Bijsterveld score of ≥4), a Schirmer test result of ≤5 mm/5 minutes without anesthesia, and an unstimulated salivary flow rate of ≤0.1 ml/minute, each scoring 1

Clinical Considerations

• Dry eye and dry mouth associated with systemic immune dysfunction are the hallmark symptoms of Sjögren's syndrome 1
• Patients with Sjögren's syndrome are at increased risk for potentially life-threatening vasculitic or lymphoproliferative disorders, and early diagnosis is crucial for proper management and monitoring 1
• The American-European Consensus Group and the ACR/EULAR classification criteria are commonly used diagnostic frameworks, but the latest criteria proposed in 2024 should be prioritized due to its recency and high quality 1

Laboratory Tests

• Schirmer test to measure tear production
• Ocular surface staining to assess corneal damage
• Salivary gland function tests
• Minor salivary gland biopsy showing focal lymphocytic sialadenitis with a focus score ≥1 per 4 mm² is considered the gold standard for diagnosis 1

Management

• Treatment options include tear supplementation and stabilization, control of inflammation of the lacrimal glands and ocular surface, and possible stimulation of tear production 1
• Topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies seem effective, while oral secretagogues are more effective for oral dryness than ocular dryness 1

From the Research

Diagnostic Criteria for Sjögren's Syndrome

The diagnostic criteria for Sjögren's syndrome involve a combination of clinical signs and symptoms, as well as specific tests, including:

• Histology: characteristic appearance of a biopsy sample from a minor salivary gland 2
• Immunology: presence of autoantibodies such as anti-SS-A 2
• Ophthalmology: ocular tests to assess dryness 3
• Salivary flow: measurement of salivary gland function 3

Classification and Diagnosis

Sjögren's syndrome can be classified as primary or secondary, depending on whether it occurs alone or in association with other systemic autoimmune diseases 4. The diagnosis is based on characteristic clinical signs and symptoms, as well as on specific tests, including salivary gland histopathology and autoantibodies 4. Recently, new classification criteria and disease activity scores have been developed primarily for research purposes, which can also be useful tools in everyday clinical practice 4.

Key Features for Diagnosis

Key features for diagnosis include:

• Dryness of the mouth and eyes resulting from involvement of the salivary and lacrimal glands 2
• Objective signs and symptoms of dryness, including a characteristic appearance of a biopsy sample from a minor salivary gland or autoantibody such as anti-SS-A 2
• Exclusions to the diagnosis include infections with HIV, human T-lymphotropic virus type I, or hepatitis C virus 2

Imaging Characteristics

Imaging characteristics of Sjögren's syndrome include:

• Salivary imaging findings seen with modern imaging modalities, such as ultrasound, MRI, and CT scans 5
• Imaging findings of the central nervous system disorders associated with Sjögren's syndrome, including the neuromyelitis optica spectrum disorder 5