title: Sjögren's syndrome with multiple cystic lesions and pulmonary arteriovenous fistulae.
abstract: A patient presented with Sjögren's syndrome associated with pulmonary multiple cystic lesions and a pulmonary arteriovenous fistulae. A histological examination of the lungs during the autopsy revealed the stenosis of the bronchiole lumens with hyperplasia of goblet cells, proliferation of smooth muscles in the inner wall of the bronchioli and retention of mucus in the airway lumens. These small airway changes were accompanied with chronic inflammatory changes of the airways in Sjögren's syndrome and led to the formation of cystic lesions via a ball-valve mechanism. Arteriovenous fistulae were situated around the cystic lesions. There may have been a correlation between the formation of the fistulae and cysts, but no mechanism was indicated in the histological findings. This report reveals that chronic inflammatory changes of the airways in Sjögren's syndrome are sufficient to cause the formation of cystic lesions.
authors.affiliation: Department of Internal Medicine, Toyama Prefectural Central Hospital, Toyama. tan-tym@umin.ac.jp
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Taniguchi
authors.forename: Atsuo
authors.initials: A
authors.lastname: Miwa
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Abo
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Demachi
authors.forename: Saburo
authors.initials: S
authors.lastname: Izumi
mesh_terms: D001164:Arteriovenous Fistula / Q000150:complications / Q000175:diagnosis*; D003560:Cysts / Q000150:complications / Q000175:diagnosis*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008168:Lung / Q000002:abnormalities* / Q000473:pathology; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis*