Primary Sjögren syndrome in childhood: report of a case and review of the literature.

Sjögren syndrome (SS) in childhood is a rare and possibly underdiagnosed condition. The purpose of this study is to report a case of primary SS (PSS) in a 4-year-old Venezuelan girl and to review the pertinent literature. The patient presented with bilateral recurrent parotid enlargement, predominantly on the right side. She did not complain of dry mouth or eyes; however, decreased stimulated salivary flow rate and positive Schirmer and rose bengal tests were obtained. Sialography, sonograms, and a computed tomography scan of the parotid glands revealed pathologic changes consistent with SS. Anti-SS-A and anti-SS-B antibodies were present. Evaluation for antibodies against cytomegalovirus, Epstein-Barr virus, and HIV rendered negative results. Histopathologic examination of incisional biopsies of the right parotid and labial minor salivary glands revealed focal periductal lymphocytic infiltrate and sialoduct ectasia. Taken together, these findings are consistent with the diagnosis of juvenile PSS. The salient features of this rare disease are summarized on the basis of a comprehensive review of the epidemiologic, clinical, and serologic findings of the previously reported cases of PSS in children.