Sjögren Syndrome is a multisystemic autoimmune disease that is heterogeneous in its presentation, course and outcome. There is no single clinical, laboratorial or radiological feature that serves as gold standard for the diagnosis and/or classification of this syndrome. The occurrence of lymphoma is known to be one of the most severe complications. We report a case of a 66-year-old female diagnosed with Sjögren Syndrome secondary to systemic lupus erythematous that presented with an enlargement of the left parotid gland consistent with the diagnosis of lymphoma confirmed with biopsy. She received chemotherapy with favorable response and today is asymptomatic with hydroxychloroquine 400mg id. This case report highlights the importance of optimal interventions and active surveillance of Sjogren Syndrome, in order to achieve an early identification of its complications and to prevent worse outcomes of this disease.
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