Primary Sjögren's Syndrome and the Therapeutic Response to Rituximab: Case Report

Abstract

The physiopathology is resumed as the lymphocytic infiltration and B cell hyperactivity around the epithelium of the affected tissues. The etiology is still unknown, although genetic and environmental factors are believed to play a very important role in the manifestation of pSS. The diagnosis and classification are made based on pre-established criteria that consider the clinical manifestations, biological markers and cytopathological alterations. The treatment is widely based on the symptoms and clinic presented by the patient, for severe extraglandular manifestations, B-cell-targeted therapies have been showing great results especially in cryoglobulinemia, vasculitis, severe parotid swelling, inflammatory arthritis, pulmonary disease and peripheral neuropathy.