Xerostomia in patients with triple A syndrome – a newly recognised finding

• DOI: 10.1007/pl00008361
• Publication Date: 2006-04-10T10:48:09Z
• Authors: Miroslav Dumić; Marinka Mravak-St...; Zvonimir Kaić; Jasenka Ille; Vesna Plavšić; Stipe Batinica; Marijana Cvitanović
• Keywords: Adult; Adolescent; Lacrimal Apparatus Diseases; Adrenal Gland Diseases; Syndrome; Xerostomia; 3. Good health; Esophageal Achalasia; achalasia; adrenal insufficiency; alacrima; autonomic neuropathy; xerostomia; 03 medical and health sciences; 0302 clinical medicine; Humans; Female; Child

Triple A syndrome is characterised by achalasia, alacrima, adrenal insufficiency and progressive neurological abnormalities including impaired autonomic nervous function. We present five patients with triple A syndrome in whom we describe xerostomia for the first time, a symptom which was presumed to be practically exclusive to Sjøgren syndrome and familial dysautonomia. Conclusion We recommend the investigation of salivation in all patients with triple A syndrome and treatment of xerostomia in order to ease swallowing. Further, our results corroborate earlier doubts that some patients with Sjøgren syndrome, especially those with the so-called "achalasia sicca" syndrome and adrenocortical insufficiency, actually had triple A syndrome. Therefore, adrenocortical function should be assessed in all patients with Sjøgren syndrome, particularly in those with difficulties in swallowing, because even latent adrenocortical insufficiency could be life-threatening for these patients in stressful situations.