title: Unusual presentation of triple A syndrome mimicking Sjögren's syndrome.
abstract: Triple A syndrome is a rare autosomal recessive disorder characterized by alacrima, achalasia, and adrenal insufficiency. Sjögren'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 Sjögren's syndrome, which leads to a challenging diagnosis.
authors.affiliation: Department of Rheumatology, Gaziantep University School of Medicine, Gaziantep, Turkey.
authors.forename: Ahmet Mesut
authors.initials: AM
authors.lastname: Onat
authors.forename: Yavuz
authors.initials: Y
authors.lastname: Pehlivan
authors.forename: Hakan
authors.initials: H
authors.lastname: Buyukhatipoglu
authors.forename: Yusuf Ziya
authors.initials: YZ
authors.lastname: Igci
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Ziya
authors.forename: Seydi
authors.initials: S
authors.lastname: Okumus
authors.forename: Cemile
authors.initials: C
authors.lastname: Arikan
authors.forename: Sibel
authors.initials: S
authors.lastname: Oguzkan
mesh_terms: D000303:Adrenal Cortex Diseases / Q000175:diagnosis*; D000328:Adult; D002648:Child; D003241:Consanguinity; D003680:Deglutition Disorders / Q000473:pathology; D003937:Diagnosis, Differential; D004931:Esophageal Achalasia / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007249:Inflammation; D007766:Lacrimal Apparatus Diseases / Q000175:diagnosis*; D018807:Polymorphism, Single-Stranded Conformational; D017422:Sequence Analysis, DNA; D012859:Sjogren's Syndrome / Q000175:diagnosis*; D013577:Syndrome