title: Cystic lung disease in Sjögren's syndrome: An observational study.
abstract: OBJECTIVE
  To analyze the prevalence, characteristics and outcome of cystic lung disease associated with Sjögren's syndrome (SS).
  
  
  METHODS
  From June 2010 to February 2015, 90 consecutive SS patients [60.1±14.8years; 88 (97.8%) female, 75 (83.3%) primary SS] had a systematic chest CT-scan. The presence of thin-walled cysts was analyzed by one experienced radiologist. Demographic data, clinical history, laboratory findings, and pulmonary function tests were extracted retrospectively from medical records.
  
  
  RESULTS
  Twenty-one (23.3%) patients had cysts on CT scan performed 40.5±54.5months after SS diagnosis. Cysts number ranged from 1 to 25 were often bilateral (52.4%) and mostly located in the middle lung zone (76.2%). Cysts were isolated (n=6, 28.6%) or associated with other lesions, including bronchiectasis (n=5, 23.8%), micronodules (n=5, 23.8%), ground-glass opacity (n=4, 19%) and/or air trapping (n=3, 14.3%). Most patients with cysts (57.1%) had no respiratory symptoms. When comparing SS patients with and without cysts, patients with cysts tended to be older (65.3±15.3 versus 58.5±14.4years, P=0.06). Smoking habits were similar in both groups. Anti-SSB antibodies were more frequently detected in patients with cysts (57.1% vs. 26.1%, P=0.02). Pulmonary function tests were normal or displayed only mild small airways obstruction and reduced diffusion capacity to carbon monoxide. Four (19%) patients with cysts had a past history of associated pulmonary disease, including interstitial lung disease. During follow-up (25.1±17.7months), no patient developed specific lung disease or lymphoproliferative disorders.
  
  
  CONCLUSIONS
  Cystic lung disease is frequent, benign, associated with anti-SSB/La antibodies and has no impact on outcome in SS.
authors.affiliation: Département de médecine interne, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France.
authors.forename: Sarah
authors.initials: S
authors.lastname: Lechtman
authors.affiliation: Département de radiologie, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France.
authors.forename: Marie-Pierre
authors.initials: MP
authors.lastname: Debray
authors.affiliation: Département de pneumologie, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France; Département hospitalo-universitaire FIRE (Fibrosis, Inflammation and Remodelling in Renal and Respiratory Diseases), université Paris Diderot, PRES Sorbonne Paris Cité, 75018 Paris, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Crestani
authors.affiliation: Département de physiologie, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Bancal
authors.affiliation: Département de pathologie, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France.
authors.forename: Muriel
authors.initials: M
authors.lastname: Hourseau
authors.affiliation: Département de médecine interne, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Dossier
authors.affiliation: Département de médecine interne, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Alexandra
authors.affiliation: Département de médecine interne, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France.
authors.forename: Marie-Paule
authors.initials: MP
authors.lastname: Chauveheid
authors.affiliation: Département de médecine interne, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France; Département hospitalo-universitaire FIRE (Fibrosis, Inflammation and Remodelling in Renal and Respiratory Diseases), université Paris Diderot, PRES Sorbonne Paris Cité, 75018 Paris, France; Inserm U1149, université Paris Diderot, laboratoire d'excellence INFLAMEX, PRES Sorbonne Paris Cité, Paris, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
authors.affiliation: Département de médecine interne, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France; Département hospitalo-universitaire FIRE (Fibrosis, Inflammation and Remodelling in Renal and Respiratory Diseases), université Paris Diderot, PRES Sorbonne Paris Cité, 75018 Paris, France; Inserm U1149, université Paris Diderot, laboratoire d'excellence INFLAMEX, PRES Sorbonne Paris Cité, Paris, France. Electronic address: karim.sacre@aphp.fr.
authors.forename: Karim
authors.initials: K
authors.lastname: Sacre
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000150:complications*; D014057:Tomography, X-Ray Computed
keywords: Cystic disease of lung; SSB antigen; Sjogren's syndrome