From the Guidelines
Testing for Sjögren's syndrome should involve a combination of clinical evaluation, blood tests, and sometimes tissue biopsy, with a high degree of suspicion in patients with clinically significant dry eye and dry mouth symptoms, as indicated by the latest guidelines 1. The diagnosis of Sjögren's syndrome begins with blood tests including antinuclear antibody (ANA), anti-Ro/SSA and anti-La/SSB antibodies, rheumatoid factor, and tests for inflammation markers like erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) 1.
Key Diagnostic Tests
- Ophthalmologic tests to assess dry eyes, including Schirmer's test and ocular surface staining
- Oral dryness evaluation through salivary flow rate measurement and salivary gland scintigraphy
- Minor salivary gland biopsy from the lower lip, considered the gold standard, showing characteristic lymphocytic infiltration
- Additional tests may include ultrasound of salivary glands and serological tests to rule out other autoimmune conditions
Importance of Early Diagnosis
Early diagnosis is crucial as it allows for proper management of symptoms and monitoring for potential complications like lymphoma, which occurs at higher rates in Sjögren's patients 1.
Comprehensive Approach
Diagnosis requires a comprehensive approach as symptoms overlap with other conditions, and no single test is definitive 1.
Latest Guidelines
The latest classification criteria for Sjögren's syndrome are based on the weighted sum of five items, including anti-SSA/Ro antibody positivity, focal lymphocytic sialadenitis, abnormal ocular staining score, Schirmer test result, and unstimulated salivary flow rate 1.
Patient Evaluation
Evaluation of patients should include symptoms of both discomfort and visual disturbance, as well as determination of the relative contribution of aqueous production deficiency and evaporative loss of tear volume 1.
Treatment Options
Treatment options for patients with dry eye secondary to Sjögren's syndrome include topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies, with oral secretagogues being more effective for oral dryness than ocular dryness 1.
From the Research
Testing for Sjögren's Syndrome
- Sjögren's syndrome is a common inflammatory rheumatological disease with a prevalence of at least 0.4% in Germany 2
- The disease has a wide variety of presentations, ranging from local involvement of exocrine glands to systemic, extraglandular involvement of multiple organs 2
- Serologic testing reveals antinuclear auto-antibodies (anti-Ro/SSA and anti-La/SSB) as well as rheumatoid factors, which are important for diagnosis 2
Diagnostic Criteria
- Primary care physicians can play a crucial role in recognizing Sjögren's syndrome in the early stages by identifying patients with the greatest probability of being diagnosed with the disease 3
- The physical examination may provide important clues to systemic involvement, such as parotid gland enlargement, skin lesions, respiratory crackles, arthritis, and neurological sensory or motor deficits 3
- Simple laboratory studies, such as the triad of cytopenia, raised erythrocyte sedimentation rate, and high serum gamma globulin levels, can be useful in reinforcing the clinical suspicion of Sjögren's syndrome 3
Immunological Parameters
- Anti-La/SSB antibodies are rarely found without concomitant anti-Ro/SSA, and their clinical significance in patients with primary Sjögren's syndrome has been poorly investigated 4
- The presence of anti-La/SSB may help in identifying a disease subset with distinct prognostic features, especially in terms of higher risk of lymphoproliferative complications 4
- Hypergammaglobulinaemia, rheumatoid factor, and lymphoma are independent variables significantly associated with anti-La/SSB positivity 4
Treatment and Management
- The Sjögren's Syndrome Foundation clinical practice guidelines recommend the use of biologic agents, management of fatigue, and inflammatory musculoskeletal pain 5
- Rituximab may be used in selected clinical settings for oral and ocular dryness and for certain extraglandular manifestations, including vasculitis, severe parotid swelling, inflammatory arthritis, pulmonary disease, and mononeuritis multiplex 5
- Future therapies must be designed to improve symptoms of dry eyes and dry mouth, extraglandular disease, and fatigue and cognitive deficits, and may involve innovative directions such as interactions with neuroscientists and neuropsychiatrists 6