From the Guidelines
The workup for Sjögren syndrome involves a comprehensive approach combining clinical evaluation, laboratory tests, and sometimes tissue biopsy, with a focus on assessing sicca symptoms, systemic manifestations, and associated autoimmune conditions, as recommended by the most recent guidelines 1. The initial assessment should include a thorough history focusing on sicca symptoms (dry eyes, dry mouth), systemic manifestations, and associated autoimmune conditions.
- Laboratory testing should include complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, and C-reactive protein to assess inflammation.
- Specific autoantibody testing is crucial, particularly anti-SSA/Ro and anti-SSB/La antibodies, which are present in 50-70% and 30-60% of patients respectively, as noted in the study by 1.
- Rheumatoid factor and antinuclear antibodies should also be checked. Ocular evaluation includes:
- Schirmer's test (measuring tear production)
- Ocular surface staining Oral assessment may involve:
- Salivary flow rate measurement
- Salivary gland scintigraphy
- Ultrasound A minor salivary gland biopsy from the lower lip is often considered the gold standard, showing focal lymphocytic sialadenitis with a focus score ≥1, as described in the study by 1. Additional testing may include:
- Immunoglobulin levels
- Complement levels (C3, C4)
- Cryoglobulins to assess for systemic involvement This comprehensive approach is necessary because Sjögren syndrome has heterogeneous presentations and can affect multiple organ systems beyond the exocrine glands, as highlighted in the guidelines by 1 and 1. The most recent and highest quality study 1 provides the best evidence for the workup and management of Sjögren syndrome, and should be prioritized in clinical practice.
From the Research
Workup for Sjogren Syndrome
The workup for Sjogren syndrome involves a combination of clinical evaluation, laboratory tests, and specialist consultations.
- Diagnosis of Sjogren syndrome is challenging and requires coordination with a team of specialists, including dentists, otolaryngologists, rheumatologists, and ophthalmologists 2.
- Laboratory tests, such as anti-Ro/SSA and anti-La/SSB antibodies, are useful in the diagnosis of primary Sjogren's syndrome (pSS) and help to identify more "active" patients 3.
- The presence of anti-La/SSB antibodies in patients with pSS and concomitant anti-Ro/SSA and salivary gland biopsy positivity may help in identifying a disease subset with distinct prognostic features, especially in terms of higher risk of lymphoproliferative complications 4.
Clinical Evaluation
The clinical evaluation of Sjogren syndrome involves assessing the patient's symptoms, medical history, and physical examination.
- Sjogren syndrome is characterized by dry eyes and dry mouth, and other organ systems may be affected in many patients 2.
- The disease can be classified as primary or secondary, with primary disease being a solitary process and secondary disease accompanying another autoimmune disease, such as rheumatoid arthritis 2.
Laboratory Tests
Laboratory tests play a crucial role in the diagnosis and management of Sjogren syndrome.
- Anti-Ro/SSA and anti-La/SSB antibodies are the hallmark antibodies in primary Sjogren's syndrome, being present in 60-70% of patients 3.
- The frequency of these antibodies in Japanese and other Oriental patients may be double that seen in American patients 5.
- The distribution of the subtypes of the anti-Ro/SSA autoantibodies may be connected to the disease type in primary/secondary Sjogren's syndrome 6.
Treatment
Treatment of Sjogren syndrome aims to relieve symptoms and prevent complications.