Unusual cancer in primary Sjögren syndrome.

Sjögren syndrome (SS) is the second most common autoimmune disease, affecting mainly middle-aged women. The disease might occur alone (primary SS) or in association with other autoimmune diseases such as rheumatoid arthritis (secondary SS). The important symptoms of SS, dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca), result from lymphocytic infiltration and destruction of the exocrine glands, particularly the salivary and lacrimal glands.1 Patients with SS have an elevated risk of developing malignant neoplasms, particularly hematologic malignancies, with most being non-Hodgkin B-cell lymphoma.2 Other cancers, such as oral cancer, breast cancer, and thymoma, might also occur in patients with SS. However, the coexistence of SS with nasopharyngeal carcinoma (NPC) has rarely been reported, with only one case involving secondary SS described in the literature to date.3 Here we describe a case of NPC in a patient with primary SS.