Diagnosis, progression and intervention in Sjogren's syndrome

Sjögren’s syndrome (SS) is a chronic inflammatory and lymphoproliferative progressive autoimmune disease. It is characterized by B cell activation and infiltration of T and B cells in the exocrine glands. Common symptoms are related to diminished lacrimal and salivary gland function. Besides keratoconjunctivitis sicca and xerostomia, patients can present with severe systemic complications, such as vasculitis and nephritis. Furthermore, 5 percent of patients with SS develop malignant B cell lymphoma during follow-up. Diagnosis of SS remains difficult, especially with regard to the oral component. Still is much unknown with regard to loss of salivary gland function and the evolution of malignant lymphoma, and there is no evidence-based intervention therapy. The main objective of the first part of this thesis was to optimize diagnostic procedures in SS with regard to histopathology, collecting of saliva, and imaging. Next, progression of salivary gland dysfunction and lymphoma development were studied. In the last part of this thesis, the effect of B cell depletion in SS with or without MALT lymphoma was evaluated.