title: Severe excessive dynamic airway collapse in a patient with primary Sjögren's syndrome.
abstract: Airway and cystic lung diseases can be observed in patients with Sjögren's syndrome. We report a case of such a patient suffering from respiratory failure due to recurrent episodes of right pneumothorax, requiring invasive mechanical ventilation. Despite thoracic drainage and adequate pneumothorax management, the patient could not be weaned from the ventilator. Fiberoptic bronchoscopy revealed severe central excessive dynamic airway collapse of the lower part of the trachea and proximal bronchi. The severity of airway collapse was maximal at the intermediate bronchus level, with a near-complete obstruction during expiration. Inspiratory and expiratory computed tomography studies confirmed the fiberoptic findings and suggested a possible expiratory posterior compression of the intermediate bronchus by parenchymal lung cysts. Stenting was considered, but the patient died from ventilator-associated pneumonia before the procedure could be performed. This case is the first description of severe central excessive dynamic airway collapse in a patient with primary Sjögren's syndrome complicated by diffuse airway and cystic lung disease.
authors.affiliation: Service de Réanimation Médicale, Hôpital Européen Georges Pompidou, Assistance Publique-Hopitaux de Paris.
authors.forename: Sophie
authors.initials: S
authors.lastname: Ismael
authors.affiliation: Service de Pneumologie, Hôpital Européen Georges Pompidou, Assistance Publique-Hopitaux de Paris.
authors.forename: Delphine
authors.initials: D
authors.lastname: Wermert
authors.affiliation: Service de Radiologie, Hôpital Européen Georges Pompidou, Assistance Publique-Hopitaux de Paris, 20 rue Leblanc.
authors.forename: Kim-Diep
authors.initials: KD
authors.lastname: Dang-Tran
authors.affiliation: Service de Réanimation Médicale, Hôpital Européen Georges Pompidou, Assistance Publique-Hopitaux de Paris.
authors.forename: Marion
authors.initials: M
authors.lastname: Venot
authors.affiliation: Service de Réanimation Médicale, Hôpital Européen Georges Pompidou, Assistance Publique-Hopitaux de Paris.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Fagon
authors.affiliation: Service de Réanimation Médicale, Hôpital Européen Georges Pompidou, Assistance Publique-Hopitaux de Paris Institut National de la Santé et de la Recherche Médicale U765, Faculté de Pharmacie and Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité, Paris, France. jldiehl@invivo.edu.
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Diehl
mesh_terms: D000402:Airway Obstruction / Q000175:diagnosis / Q000209:etiology*; D001999:Bronchoscopy; D004322:Drainage; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D008875:Middle Aged; D011030:Pneumothorax / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D012121:Respiration, Artificial; D012129:Respiratory Function Tests; D012859:Sjogren's Syndrome / Q000150:complications*; D014057:Tomography, X-Ray Computed
keywords: Sjögren's syndrome; bronchoscopy; expiratory dynamic airway collapse; inspiratory and expiratory ct; lung cysts; respiratory failure