Unusual presentation of triple A syndrome mimicking Sjogren's syndrome

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

CLINICAL RHEUMATOLOGY, cilt.26, sa.10, ss.1749-1751, 2007 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 26 Sayı: 10
  • Basım Tarihi: 2007
  • Doi Numarası: 10.1007/s10067-006-0498-5
  • Dergi Adı: CLINICAL RHEUMATOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.1749-1751
  • Anahtar Kelimeler: Sjogren's syndrome, triple a syndrome, NUCLEAR-PORE COMPLEX, WD-REPEAT PROTEIN, ACHALASIA, INSUFFICIENCY, DEFICIENCY, MUTATIONS, AAAS, GENE
  • Bursa Uludağ Üniversitesi Adresli: Hayır

Özet

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.