Abstract
Sjögren’s syndrome (SjS) is very rare in childhood. Secondary SjS followed by systemic lupus erythematosus sometimes occurs; however, this disease followed by juvenile idiopathic arthritis (JIA) has only rarely been reported. We encountered a young woman with juvenile SjS who developed rheumatoid arthritis (RA) at the age of 19 years, 9 years after the onset of SjS.
At age 10, this patient visited our hospital with low-grade fever, erythema, lymphadenopathy, dryness of mouth, arthralgia, and general fatigue. Laboratory examinations showed elevated serum total protein, high serum IgG, positive autoantibodies such as antinuclear antibody, rheumatoid factor, anti-Ro/SS-A antibody, and anti-La/SS-B antibody. Histopathological and sialographic changes in salivary glands and hypofunction of the lacrimal glands were also revealed. We diagnosed the patient with primary SjS and treated her with predonisolone, mizoribin, and methotrexate thereafter.
Nine years after the onset of SjS, the patient developed arthralgia and swelling of the finger joints, and arthritis extended to the joints of both hips, the right knee, and the right shoulder with morning stiffness. In laboratory findings, C-reactive protein, erythrocyte sedimentation rare, and serum matrix metalloproteinase (MMP)-3 were elevated. The diagnosis of RA is confirmed by those findings. She was considered to have secondary SjS with RA based on the course of diagnosis.
In a juvenile SjS patient, the symptoms of RA morbidity may develop with delayed onset. In the patient diagnosed with JIA, they may have overlooked the morbidity of SjS.
