Classification criteria and treatment modalities in primary Sjögren’s syndrome

Abstract

Primary Sjögren’s syndrome is a systemic autoimmune disease, characterized by a lymphocytic exocrinopathy. Oral and ocular dryness, asthenia and pain represent hallmarks of the disease. Systemic manifestations concern a third of patients, including lymphoma in 5% of the patients. The American European Consensus Group classification criteria have been used in current practice and clinical trials since 2002. New classification criteria were recently proposed by the American Congress of Rheumatology. A group of international experts are currently working to reach a new consensus between the American European Consensus Group classification criteria and the American Congress of Rheumatology proposal for disease classification. In addition, international consensus disease activity scores were recently established. Regarding treatment modalities, symptomatic treatments remain the cornerstone of therapy in pSS, but new biologic treatments are currently evaluated.

Keywords::

• abatacept
• ACR criteria
• AECG criteria
• belimumab
• biologics
• ESSDAI
• ESSPRI
• gene therapy
• Primary Sjögren’s syndrome
• rituximab
• tocilizumab

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Sjögren’s syndrome (SS) is a systemic autoimmune disease characterized by salivary and lachrymal gland dysfunction leading to dry mouth and eyes. Systemic involvement concerns about a third of patients.

• The American European Consensus Group criteria, used in current practice, take into account subjective and objective clinical parameters, as well as immunological and histological criteria.

• New criteria were proposed by the American Congress of Rheumatology, which take into account objective ocular dryness, salivary lymphocytic infiltrates and the presence of immunological abnormalities.

• A group of international experts is currently working on the development of a new consensus classification criteria.

• The consensus international scores, the Eular SS disease activity index and the Eular SS patient-related index allow to evaluate disease activity by clinicians and patients and could be useful as primary outcome criteria in clinical trials.

• The arsenal of biologic therapies of potential interest to treat primary SS is expanding as a result of the better understanding of the pathogenesis of the disease. These drugs deserve to be evaluated in controlled trials.

• B-cell-activating factor of the TNF family with other cytokines such as IL-6 and IL-21, which are powerful activators of B lymphocytes, could notably represent therapeutic targets of interest.

Notes

According to these criteria, a patient can be classified as having Sjögren’s syndrome, when there are at least two out of the three above criteria.

Data taken from [3].

Data taken from [3].