A Turkish family with xerostomia

Xerostomia is an important symptom which disrupts the quality of life very seriously, and may be the major symptom of Sjogren’s syndrom (SS). The etiology for SS remains unknown where multifactorial influences contribute to the pathogenesis of subsequent development of the disease.
In this letter we report a Turkish family with xerostomia whose genetic susceptibility to SS followed a mendelian dominant trait. This family contain one grandfather (age: 70), 5
children (age: 20-36) and 6 grandchidren (age: 5-9). Grandfather, four of five chidren and two of six grandchidren had xerostomia. Seven member of the family reported the following: Inability to eat dry food, putting a glass of water on their bed stand to drink at night, difficulty speaking for long periods of time, higher incidense of dental caries and periodontal disease. All cases stated that his complaints of dry mouth was
started at the age of 3 after a common cold. Physical examination revealed dental caries and periodontal disease. The routine laboratory tests were within normal limits and RF,
ANA, SS-A (anti-RO), SS-B (anti-LA) was negative. In all patients we found those an unstimulated whole mouth salive flow rate of < 0.1 ml/min, Rose Bengal test was positive and Schirmer test was negative. Glandulary stricture and inflamatory cells wasn’t seen in labial biopsy. Our patient had two of Fox criteria.
It seems to be a genetic predisposition for the SS in some cases. Immunogenetic studies have demonstrated that HLA-B8, - DR-3, and –DRw52 are prevalent in primary Sjogren’s syndrome patients as compared with the normal control population.
On the other hand, family studies indicated the presence of an additional autosomal dominant gene(s), not linked to HLA, in predisposition to SS. Possesion of the necessary genetic factors doesn’t invariably lead to clinical disease. The cumulative effect of interactive genetic (MHC and non MHC) and nongenetic influences (virus infection) may dictate protective and/or susceptibility factors in disease expression. In conclusion, xerostomia may show familial occurens and other family members are asked for this symptom.

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