Unusual presentation of triple A syndrome mimicking Sjogren's syndrome


Onat A. M. , Pehlivan Y. , Buyukhatipoglu H., Ziya Y., Okumus S., Arikan C., ...More

CLINICAL RHEUMATOLOGY, vol.26, no.10, pp.1749-1751, 2007 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 26 Issue: 10
  • Publication Date: 2007
  • Doi Number: 10.1007/s10067-006-0498-5
  • Title of Journal : CLINICAL RHEUMATOLOGY
  • Page Numbers: pp.1749-1751
  • Keywords: Sjogren's syndrome, triple a syndrome, NUCLEAR-PORE COMPLEX, WD-REPEAT PROTEIN, ACHALASIA, INSUFFICIENCY, DEFICIENCY, MUTATIONS, AAAS, GENE

Abstract

Triple A syndrome is a rare autosomal recessive disorder characterized by alacrima, achalasia, and adrenal insufficiency. Sjogren's syndrome (SS) is a chronic inflammatory disorder manifested primarily by diminished lacrimal and salivary gland secretions, resulting in symptoms of dry eyes and dry mouth, the so-called "sicca complex". However, a variety of other manifestations also can occur, which can be termed "nonexocrine manifestations". One of the frequent nonexocrine manifestations is dysphagia. In this paper, we present an unusual form of Triple A disease mimicking Sjogren's syndrome, which leads to a challenging diagnosis.