From the Guidelines
Sjögren's syndrome with a positive Anti-Extractable Nuclear Antigen (ENA) titer is an autoimmune disorder characterized by inflammation and dysfunction of exocrine glands, particularly the salivary and lacrimal glands, leading to symptoms of dry eyes and dry mouth. The presence of a positive ENA titer, specifically anti-SSA/Ro antibodies, is a key diagnostic criterion for Sjögren's syndrome, as noted in the 2020 EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies 1.
Key Points
- A positive ENA titer indicates immune system activity against self-proteins and supports the diagnosis of Sjögren's syndrome, especially when combined with clinical symptoms and other diagnostic criteria.
- Management typically includes artificial tears and saliva substitutes for symptom relief, along with systemic medications like hydroxychloroquine (200-400mg daily) for more severe manifestations, as recommended by the 2020 EULAR guidelines 1.
- Regular monitoring is essential as patients with Sjögren's syndrome have an increased risk of developing lymphoma and other complications, as highlighted in the 2024 dry eye syndrome preferred practice pattern 1.
- The presence of these antibodies may also indicate a more systemic disease process affecting multiple organs beyond the exocrine glands, as discussed in the 2024 dry eye syndrome preferred practice pattern 1.
Diagnostic Criteria
- 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, as outlined in the 2024 dry eye syndrome preferred practice pattern 1.
- A total score of ≥4 for these items meets the criteria for primary Sjögren's syndrome.
Treatment Options
- Topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies seem effective for dry eye secondary to Sjögren's syndrome, as noted in the 2024 dry eye syndrome preferred practice pattern 1.
- Oral secretagogues, such as pilocarpine and cevimeline, may be effective for sicca features, while topical anti-inflammatories, such as cyclosporine, may be beneficial for moderate or severe dry eye, as discussed in the 2020 EULAR recommendations 1.
Ongoing Research
- The current therapeutic pipeline in Sjögren's syndrome is shifting towards the evaluation of biologics targeting cytokines, T-cells, and intracellular signalling pathways, as highlighted in the 2020 EULAR recommendations 1.
- Ongoing trials are testing the effect of monoclonal antibodies targeting BAFF-receptor or the association between B-cell depletion and BAFF inhibition, as well as other pathways activating B cells, as noted in the 2020 EULAR recommendations 1.
From the Research
Definition of Sjögren's Syndrome
Sjögren's syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration and malfunction of the exocrine glands, resulting in dry mouth and eyes 2. It can present either alone (primary Sjögren's syndrome) or in the context of an underlying connective tissue disease (secondary Sjögren's syndrome) 2, 3.
Autoantibodies in Sjögren's Syndrome
The detection of autoantibodies, such as anti-Ro/SSA and anti-La/SSB, is important for disease classification and diagnosis 4. Novel autoantibodies, including anti-SP-1, anti-CA6, and anti-PSP, have been identified in Sjögren's syndrome and may be useful in identifying particular stages of the disease or predicting long-term complications 4.
Clinical Parameters of Sjögren's Syndrome
Clinical parameters of Sjögren's syndrome include xerostomia and keratoconjunctivitis sicca, resulting from lymphocytic infiltration of salivary and lacrimal glands 3. Systemic features, such as cutaneous, respiratory, renal, hepatic, neurologic, and vascular involvement, can also occur 2.
Immunological Parameters of Sjögren's Syndrome
Immunological parameters of Sjögren's syndrome include the deposition of autoantibodies, such as anti-SS-A (anti-Ro) and anti-SS-B (anti-La) 3. T cells and B cells are involved in the pathogenesis of Sjögren's syndrome, with Th1, Th2, Th17, follicular helper T (Tfh) cells, and regulatory cells (Tregs/Bregs) playing a role 3.
Anti-Extractable Nuclear Antigen (ENA) Titer
There is no direct information available on the specific role of a positive Anti-Extractable Nuclear Antigen (ENA) titer in Sjögren's syndrome in the provided studies. However, it is known that autoantibodies, including anti-ENA, are commonly found in autoimmune diseases and can be used as diagnostic markers 4.
- Key features of Sjögren's syndrome include:
- Lymphocytic infiltration of exocrine glands
- Malfunction of exocrine glands
- Dry mouth and eyes
- Systemic features, such as cutaneous, respiratory, renal, hepatic, neurologic, and vascular involvement
- Autoantibodies, including anti-Ro/SSA and anti-La/SSB, are important for disease classification and diagnosis
- Novel autoantibodies, including anti-SP-1, anti-CA6, and anti-PSP, may be useful in identifying particular stages of the disease or predicting long-term complications