SICCA MANIFESTATIONS IN SJÖGREN'S OVERLAP SYNDROME: A CASE REPORT

Primary Sjögren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands and results in the severe dryness of mucosal surfaces, mainly the mouth and eyes due to inflammation and resultant pathology of lacrimal or salivary glands.1,2 This disease predominantly affects middle-aged women, but can also be observed in children,men and the elderly1.One-half of affected individuals also develop extra-glandular involvement implying the occurrence of signs and symptoms in organs other than the salivary and lacrimal glands including the joints,skin,lungs, gastrointestinal (GI) tract,nervous system,and kidneys.3 Sjögren syndrome frequently occurs in conjunction with other autoimmune disorders including rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) which is also known as Secondary Sjögren's or Sjögren's Overlap Syndrome.4 The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease and lymphoma.3,5 The pathophysiology of SjS is the destruction of the epithelium of the exocrine glands,as a consequence of abnormal B cell and T cell responses to the autoantigens such as Ro/SSA and La/SSB,among others.6,7 Diagnostic criteria for SjS include the detection of autoantibodies in patient serum and histological analysis of biopsied salivary gland tissue.4,9 Therapeutic approaches for SjS include both topical and systemic treatments to manage the sicca and systemic symptoms of disease.5,10 SjS is a serious disease with excess mortality, mainly related to the systemic involvement of disease and the development of lymphomas in some patients. Knowledge of SjS has progressed substantially, but this disease is still characterized by sicca symptoms, the systemic involvement of disease, lymphocytic infiltration to exocrine glands, the presence of anti-Ro/SSA and anti-La/SSB autoantibodies and the increased risk of lymphoma in patients with SjS.3,5