A case of primary Sjögren's syndrome in a young girl whose initial manifestation was high fever

We herein report a case of primary Sjögren's syndrome in a young girl whose initial manifestation was high fever. A 16-year-old girl had previously experienced two episodes of short-term hospitalization at a nearby hospital due to a high fever in 1990. Since her serum anti-nuclear antibody was strongly positive, she was suspected of having unclassified connective tissue disease (UCTD) and thus was observed without medication at our outpatient clinic for two years. During this period, she only demonstrated several episodes of low grade fever when under stress such as during examinations at her school. In February 1992, she was admitted to a nearby hospital complaining of high fever, chills and polyarthralgia. Although these symptoms improved soon after the administration of oral prednisolone (30 mg/day), she was transferred to our hospital for further examination. Although she did not show any past history of either salivary gland swelling or sicca symptoms, both sialography and a labial gland biopsy were performed because the antibody to SS-A/SS-B proved positive in her serum. These examinations resulted in a definite diagnosis of primary Sjögren's syndrome. Antibodies to SS-A/SS-B were retrospectively identified to be positive in her serum during the first episode of high fever in 1990. This indicates that she was already affected by Sjögren's syndrome in back 1992 when she was 16 years old. Few cases of primary Sjögren’ s syndrome have been reported in patients younger than 16 years old whose initial manifestation was only a high fever. It is thus considered likely that more cases of primary Sjögren’ s syndrome could potentially exist in young patients with a fever of unknown origin or UCTD based on an evaluation of serum SS-A/SS-B antibodies, sialography and a labial gland biopsy.