Sj�gren's syndrome in a child

Abstract

Sjögren's syndrome is a relatively uncommon condition in the paediatric age group. The youngest child reported thus far was a 5-year-old girl. This article reports the case of a 2-year-old girl admitted for recurrent infections of the respiratory tract with diffuse pulmonary interstitial infiltrations and a progressive swelling of the parotid glands. The clinical features and the results of laboratory investigations, including parotid and hepatic biopsies, chest X-rays and sialography helped to establish the diagnosis of Sjögren's syndrome. Clinical, immunological and genetic characteristics of Sjögren's syndrome are reviewed.

Sjogren syndrome in childhood: report of two cases

“…Presence of antibodies to Ro (SSA) cellular antigen, common in adults with I~ [8] has not been reported in children with the disease and was found in our two patients. Serious extraglandular manifestations of SS also seem to be uncommon in children since they have not been previously reported [3,4,13] and were not found in our patients. On the other hand, it is well known that susceptibility to I~ has been associated with HLA-DR3 and to 2~ with HLA-DR4 in rheumatoid arthritis and HLA-DRw52 with both [7].…”

“…The disease in children is uncommon, having been rarely reported [3,4,13]. We describe two additional children with this, rare but potentially serious disease.…”

“…In previous report's it has been emphasized that the disease can start before menarche [3,4,13], and that the commonest manifestation in these patients is intermittent parotid gland enlargement [2,3]. In this age group the disease has been reported to progress slowly and to take approximately 3 years for development of the full clinical picture [3].…”

“…Although recurrent respiratory tract infections have been described as initial manifestations of SS [13], monilial stomatitis and vulgovaginitis have not. Infections or infestations can occur in these patients, particularly in mucosae devoid of normal secretions.…”

Primary Sjögren syndrome in the paediatric age: a multicentre survey

“…SS most commonly affects middle-aged women and has been described very rarely in childhood, especially in its primary form [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,23,24,25]. Our series is one of the largest published up to now on primary SS in childhood.…”