CASE REPORTS - Primary and secondary Sjogren's syndrome in children - a comparative study

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Clin Oral Investig. 2000 Sep;4(3):176-82.

Primary and secondary Sjogren's syndrome in children--a comparative study.

Stiller M, Golder W, Doring E, Biedermann T.

Department of Dental, Oral and Maxillofacial Surgery, Dental Surgery and

Radiology Section, lin Hospital, Free University of Berlin,

Assmannshauser Str. 4-6, 14197 Berlin, Germany. mstiller@...

Sjogren's syndrome is a chronic inflammatory systemic autoimmune disease

mainly affecting the exocrine and, particularly, the salivary and lacrimal

glands. The condition usually occurs in adults. In 1994, the criteria for

this syndrome were redefined in a multicenter European study. In children,

Sjogren's syndrome is a rare and probably underdiagnosed disease. To date,

Sjogren's syndrome in children has only been described in case reports and

in the comparative presentation of various study results. So far, no study

of a comparative classification into primary and secondary Sjogren's

syndrome has been carried out in a patient population of any size. Sjogren's

syndrome should be considered in the differential diagnosis of children with

recurrent parotitis, keratoconjunctivitis sicca, or pronounced and early

tooth decay associated with xerostomia. In this study of 23 children and

adolescents under the age of 16 with the clinical symptoms and laboratory

findings of Sjogren's syndrome, we differentiate between primary and

secondary Sjogren's syndrome. The value of the individual methods of

assessing the oral and the ophthalmological components and the manifestation

of the underlying rheumatic condition are discussed on the basis of the

EULAR criteria. The EULAR diagnostic criteria are of limited applicability

in children because reliable anamnestic data are frequently lacking. Another

problem in diagnosing Sjogren's syndrome is the short-term detection of

serological alterations and clinical symptoms. Even if young patients do not

completely fulfill the required criteria, Sjogren's syndrome can be assumed

or confirmed in the presence of positive testing for oral and ocular

manifestations and recurrent salivary gland enlargement.

PMID: 11000325

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve & db=PubMed & list_uids=1\

1000325

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