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

From the Guidelines

Testing for Sjögren's syndrome should involve a combination of blood tests, including anti-SSA (Ro) and anti-SSB (La) antibodies, rheumatoid factor, and antinuclear antibodies, as well as a minor salivary gland biopsy and functional tests such as the Schirmer test and sialometry, as recommended by the most recent study 1. The diagnosis of Sjögren's syndrome is complex and requires a multifaceted approach. The condition is characterized by dry eye and dry mouth associated with systemic immune dysfunction, and about 10% of patients with clinically significant dry eye have an underlying Sjögren's syndrome 1. Some key points to consider in the diagnosis of Sjögren's syndrome include:

• Blood tests to check for specific antibodies, particularly anti-SSA (Ro) and anti-SSB (La) antibodies, which are present in many Sjögren's patients 1
• Rheumatoid factor, antinuclear antibodies (ANA), and inflammatory markers like ESR and CRP are also typically measured 1
• A minor salivary gland biopsy is often performed to examine for characteristic inflammatory changes 1
• Functional tests, such as the Schirmer test to measure tear production and sialometry to assess saliva production, are also important 1
• Imaging studies, such as salivary gland ultrasonography or sialography, may be used to evaluate gland structure 1
• Eye examinations, including ocular surface staining with dyes, help assess corneal damage from dryness 1 It is essential to note that Sjögren's syndrome is an autoimmune condition that can affect multiple organs beyond just causing dry eyes and mouth, and early diagnosis allows for appropriate management of symptoms and monitoring for potential complications like lymphoma, which occurs at higher rates in Sjögren's patients 1. The latest classification criteria for Sjögren's syndrome are based on the weighted sum of five items, including anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis, and individuals with a total score of ≥4 meet the criteria for primary Sjögren's syndrome 1. In terms of treatment, topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies seem effective, although there is a paucity of rigorous clinical trials to support therapy recommendations 1. Overall, a comprehensive diagnostic approach is necessary to confirm the presence of Sjögren's syndrome and to guide appropriate management and treatment.

From the Research

Sjögren's Disease Testing

• Sjögren's syndrome is a systemic autoimmune disease characterized by immune-mediated injury of exocrine glands, as well as a diverse array of extraglandular manifestations 2.
• The detection of autoantibodies is important for the diagnosis and classification of Sjögren's syndrome, with anti-Ro/SSA and anti-La/SSB antibodies being traditional biomarkers 2, 3.
• Novel autoantibodies, such as anti-SP-1, anti-CA6, and anti-PSP, have been identified in Sjögren's syndrome patients and may be useful in identifying particular stages of the disease or predicting long-term complications 2.
• The presence of anti-Ro/SSA and anti-La/SSB autoantibody-producing cells in salivary gland biopsy tissues from patients with Sjögren's syndrome has been demonstrated, indicating their potential involvement in the autoimmune exocrinopathy of the disease 3.

Diagnostic Tools

• Salivary gland biopsy is a useful diagnostic tool for Sjögren's syndrome, with the presence of autoantibody-producing cells in glandular biopsy tissues being correlated with serum autoantibody levels 3.
• The detection of anti-La/SSB antibodies in primary Sjögren's syndrome patients with combined positivity for anti-Ro/SSA and salivary gland biopsy may help identify a disease subset with distinct prognostic features, especially in terms of higher risk of lymphoproliferative complications 4.
• Autoantibodies, such as cryoglobulins, anti-centromere antibodies, and anti-mitochondrial antibodies, are associated with significant clinical phenotypes in Sjögren's syndrome patients and can be used to categorize patients into subgroups according to their serological profile 5.

Clinical Presentation

• Sjögren's syndrome is characterized by dry eyes and dry mouth, with other organ systems being affected in many patients 6.
• 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 6.
• The clinical presentation of Sjögren's syndrome can vary widely, with some patients experiencing mild symptoms and others experiencing more severe symptoms, such as lymphoproliferative disorders 4.