Primary Sjögren Syndrome in a Two-Year-Old Patient

Primary Sjögren syndrome (pSS) is a rare autoimmune disease, especially in children, mainly affecting girls (77%), and usually diagnosed around age 10 years. Differential conditions should be considered, especially the ones that promote xerostomia, and conditions associated with parotid enlargement should also be excluded. In this case report article by Marcio Augusto de Oliveira et al, a pediatric female patient age 2 years complaining about dry mouth, oral discomfort, and dysphagia was referred for dental treatment. The patient presented 5 of the required criteria to establish the diagnosis of pSS, including ocular symptoms, oral symptoms, evidence of keratoconjunctivitis sicca, focal sialadenitis confirmed by minor salivary gland biopsy, and evidence of major salivary gland involvement. The patient did not have positive SS-A and SS-B autoantibodies. According to the literature, about 29% of individuals with pSS can present seronegativity for SS-A (anti-Ro) antibodies and about 33% can present seronegativity for SS-B (anti-La) antibodies.
To the best of the authors' knowledge, this is the youngest patient reported in the scientific English literature with pSS. Primary Sjögren syndrome has a wide clinical and immunologic spectrum and may progress with increased morbidity. Clinicians must be aware of the development of pSS in such an early age and exclude all possible differential findings to provide early diagnosis and treatment.
(Source: International Journal of Paediatric Dentistry, doi: 10.1111/j.1365-263X.2011.01140.x; this article was first published online May 30, 2011)