title: Tracheobronchial amyloidosis in a patient with Sjogren syndrome.
abstract: Pulmonary amyloidosis in the setting of Sjogren syndrome is rare. It most commonly presents in form of multinodular disease with or without cysts formation. Amyloid plaques may also deposit in the airway submucosa, causing airway narrowing; the condition referred as "tracheobronchial amyloidosis" (TBA). Patients with this condition most commonly present with postobstructive pneumonia, wheezing, and occasionally hemoptysis. Endoscopic therapies, using flexible forceps with or without laser therapy is crucial to alleviate the obstruction and control the bleeding. Other therapeutic modalities such as external beam radiation has been shown to be promising; however, further data are still needed. To our knowledge, our patient is the first reported case of TBA in a setting of Sjogren syndrome.
authors.affiliation: Department of Surgery, Harvard Medical School, Beth Israel Deaconess Medical Center, Division of Thoracic Surgery and Interventional Pulmonology, Boston, MA 02215, USA.
authors.forename: Hanine
authors.initials: H
authors.lastname: Inaty
authors.forename: Erik
authors.initials: E
authors.lastname: Folch
authors.forename: Christopher
authors.initials: C
authors.lastname: Stephen
authors.forename: Adnan
authors.initials: A
authors.lastname: Majid
mesh_terms: D000686:Amyloidosis / Q000150:complications* / Q000628:therapy; D001982:Bronchial Diseases / Q000150:complications* / Q000628:therapy; D001999:Bronchoscopy; D003646:Debridement / Q000379:methods; D006801:Humans; D008171:Lung Diseases / Q000150:complications / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000150:complications*; D014133:Tracheal Diseases / Q000150:complications* / Q000628:therapy