i111 Sjögren’s syndrome and current management

Abstract

Sjögren's syndrome is a chronic, immune-mediated, condition of unknown aetiology characterised by focal lymphocytic infiltration of exocrine glands. It typically affects women in their 40's and above, although up to 10% of cases occur in men and it is also seen in younger people. The prevalence in women in the UK has been estimated at 0.1-0.4%. Patients classically complain of drying of the eyes and mouth along with fatigue and joint pain and there is often a long delay between symptom onset and diagnosis. Other features include salivary gland swelling, joint inflammation, skin rashes, neuropathies, interstitial lung disease and a 5-10% lifetime risk of Bcell lymphoma. There is an association with autoimmune thyroid disease, coeliac disease and primary biliary cirrhosis. Systemic features include inflammatory arthritis, subacute lupus erythematosus, and immune thrombocytopaenia, vasculitis with purpura, salivary. The management of the glandular features includes conserving, replacing and stimulating secretions. Systemic features may require specific therapy and immunomodulatory treatment. Holistic management is important and many patients benefit from non-pharmacological therapies and general support. Newer, more targeted therapies are in development and may change the future management of the condition.