Casi clinici di due bambini con sindrome di Sjögren primaria

Abstract

Sjogren-Larsson syndrome (SLS) is a chronic autoimmune disease of unknown etiology most commonly seen in women (male/female ratio 1:9) mainly in the fourth and fifth decades of life. It may occur alone (primary Sjogren syndrome) or in association with other autoimmune diseases (secondary Sjogren syndrome) such as rheumatoid arthritis, systemic lupus erythematosus or systemic sclerosis. We report two cases of SLS. The first case report is about a 10-year-old girl with a history of episodes of tumor, calor and rubor in the back of her toes almost every month, which resolved in 4–5 days without therapy. She did not complain of dry mouth or eyes. The laboratory findings showed high inflammation markers, rheumatoid factor 128 IU, Waaler-Rose 256 IU, anti nuclear antibody (ANA) 1/640, SSA (anti-Sjogren antigen A) and SSB (anti-Sjogren antigen B) positive and hypergammaglobulinemia. The Schirmer’s test resulted pathologic, the ultrasonography images and biopsy of salivar glands and minor salivary glands revealed focal periductal lymphocytic infiltrate and sialoduct ectasia class IV of juvenile Sjogren syndrome. The second case report describes a 7-year-old boy with enlargement of both parotid glands and fever which occurred twice a month, treated with antibiotics and adenotonsillectomy. In his family, his maternal aunt suffered from Sjogren syndrome and his mother was under investigation for suspected Sjogren syndrome. Under our observation the child showed rheumatoid factor 37 IU, ANA 1/1280, SSA and SSB positive. He didn’t complain of dry mouth or eyes but the eye examination showed signs of keratoconjuctivitis sicca. The ultrasonography images of salivary glands and parotids revealed chronic inflammation. Juvenile Sjogren syndrome is uncommon in children. Laboratory findings may be similar to those found in adults and it may be important to consider also familiar history and other clinical manifestations, which might be different from the adult form.