Sarah Lechtman1, Marie-Pierre Debray2, Bruno Crestani3, Catherine Bancal4, Muriel Hourseau5, Antoine Dossier1, Jean-François Alexandra1, Marie-Paule Chauveheid1, Thomas Papo6, Karim Sacre7. 1. Département de médecine interne, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France. 2. Département de radiologie, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France. 3. 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. 4. Département de physiologie, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France. 5. Département de pathologie, hôpital Bichat, université Paris Diderot, PRES Sorbonne Paris Cité, Assistance publique-Hôpitaux de Paris, 75018 Paris, France. 6. 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. 7. 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.
Abstract
OBJECTIVES: 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.
OBJECTIVES: 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: Gabriel Maciel; Luisa Servioli; Carlotta Nannini; Alvise Berti; Cynthia S Crowson; Sara J Achenbach; Eric L Matteson; Divi Cornec Journal: RMD Open Date: 2018-02-28
Authors: Augustine S Lee; R Hal Scofield; Katherine Morland Hammitt; Nishant Gupta; Donald E Thomas; Teng Moua; Kamonpun Ussavarungsi; E William St Clair; Richard Meehan; Kieron Dunleavy; Matt Makara; Steven E Carsons; Nancy L Carteron Journal: Chest Date: 2020-10-16 Impact factor: 9.410