Primary Sjogren's syndrome in children and adolescents: Proposal for diagnostic criteria

Objective Primary Sjogren's syndrome (pSS) in childhood is a rare disease. Diagnostic criteria are available for adult patients only. In order to establish diag nostic criteria for juvenile pSS an analysis of 7 girls and one boy sufferi ng from pSS with early onset is reported. Due to the rarity of the disease, data on patients with pSS reported in the literature are included in the p roposal for modified diagnostic criteria. Methods The diagnosis of pSS was established according to the criteria for adulthoo d pSS, duly modified, which include clinical symptoms and laboratory immuno logical evaluation. Results The average age of our patients at clinical onset was 13.5 years (range: 10 - 17 yrs.). Clinical signs included systemic (fever, fatigue) as well as l ocal (parotitis, vulvovaginitis, conjunctivitis) symptoms. Paralysis due to hypokalemia linked to renal tubular acidosis and central nervous system (C NS) involvement was seen in one patient. Asymptomatic renal tubular acidosi s was diagnosed in another 2 patients. Autoimmune hepatitis was present in 2 patients. All patients had laboratory abnormalities: hyperimmunoglobuline mia IgG, high titers of antinuclear antibodies (anti-SS-A and/or anti-SS-B) and elevated ser um amylases. Sicca syndrome was never seen during childho od, although it developed later in 3 patients, after 7 to 10 years of follo w-up. Conclusion It has been stressed that the classical diagnostic criteria for adult Sjogr en's syndrome, especially sicca syndrome, are not applicable to a pediatric onset of the disease. On the other hand, the presence of typical laborator y abnormalities can allow the diagnosis of these patients in the early stag es. Both laboratory and clinical symptoms typical for childhood are include d in our proposal for diagnostic criteria applicable to juvenile pSS. Life- threatening conditions such as hypokalemic paralysis, CNS involvement and h epatitis may also occur in children. Sicca syndrome tends to develop much l ater in pediatric patients.