title: FEV1 over time in patients with connective tissue disease-related bronchiolitis.
abstract: BACKGROUND
  Fibrosis or inflammation of the bronchioles is a well-known manifestation of connective tissue disease (CTD). However, the natural history of CTD-related bronchiolitis is largely unknown.
  
  
  METHODS
  We analyzed consecutive patients evaluated at National Jewish Health (Denver, CO) from 1998 to 2008 with CTD and surgical lung biopsy-confirmed bronchiolitis. Linear mixed effects models were used to estimate the longitudinal postbronchodilator FEV1 %predicted (%pred) course and differences between subjects with or without constrictive bronchiolitis (CB).
  
  
  RESULTS
  Of 28 subjects with a mean age of 53 ± 9 years, fourteen (50%) had CB. The most common CTD diagnosis was rheumatoid arthritis (n = 14; 50%). There were no significant differences in demographics, smoking status, underlying CTD diagnoses, 6-min walk distance, dyspnea score or drug therapy between subjects with CB and those with cellular bronchiolitis. Three subjects with CB (11%) and four with cellular bronchiolitis (14%) died. Compared with subjects with CB, those with cellular bronchiolitis had higher mean FEV1 %pred at all times. There were no significant differences in FEV1 %pred slope within- or between-groups (CB vs. cellular bronchiolitis) preceding surgical lung biopsy or afterward.
  
  
  CONCLUSIONS
  Subjects with CTD-related CB had lower FEV1 %pred values than those with CTD-related cellular bronchiolitis at all time points, but FEV1 %pred remained stable over time in both groups regardless of therapy received.
authors.affiliation: Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO 80206, USA. Fernandezevans@njhealth.org
authors.forename: Evans R
authors.initials: ER
authors.lastname: Fernández Pérez
authors.forename: Mahalakshmi
authors.initials: M
authors.lastname: Krishnamoorthy
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
authors.forename: Tristan J
authors.initials: TJ
authors.lastname: Huie
authors.forename: Aryeh
authors.initials: A
authors.lastname: Fischer
authors.forename: Joshua J
authors.initials: JJ
authors.lastname: Solomon
authors.forename: Richard T
authors.initials: RT
authors.lastname: Meehan
authors.forename: Amy L
authors.initials: AL
authors.lastname: Olson
authors.forename: Rosane Duarte
authors.initials: RD
authors.lastname: Achcar
authors.forename: Jeffrey J
authors.initials: JJ
authors.lastname: Swigris
mesh_terms: D000328:Adult; D001706:Biopsy; D001989:Bronchiolitis Obliterans / Q000209:etiology / Q000401:mortality / Q000473:pathology / Q000503:physiopathology*; D003240:Connective Tissue Diseases / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume / Q000502:physiology*; D006801:Humans; D008168:Lung / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests / Q000379:methods; D012189:Retrospective Studies; D012737:Sex Factors; D014481:United States / Q000453:epidemiology; D014797:Vital Capacity / Q000502:physiology