title: Pulmonary involvement in long-term mixed connective tissue disease: functional trends and image findings after 10 years.
abstract: OBJECTIVE
  Interstitial lung disease (ILD) is highly prevalent in patients with mixed connective tissue disease (MCTD). However, little is known about the long-term progression of ILD in MCTD. The aims of this study were to describe pulmonary function test (PFT) and high-resolution computed tomography (HRCT) results in long-term MCTD patients, to measure changes in PFT and HRCT results over a 10-year period, and to ascertain correlations in functional and imaging data.
  
  
  METHODS
  In this retrospective cohort study, comparison between baseline and follow-up PFT and HRCT data was performed for 39 unselected consecutive MCTD patients.
  
  
  RESULTS
  At baseline, 51% of the patients had abnormal PFTs. Forced vital capacity (FVC) was slightly reduced at baseline (77% of predicted), but remained stable after 10 years. A relative decrease of 15% in the diffusion capacity for carbon monoxide (DLCO) was detected (from 84% to 71% of predicted, p<0.001). The median lower lobes ILD-HRCT score progressed from 7.5% at baseline to 11.2% at follow-up (p=0.02), and findings of traction bronchiolectasis and honeycombing increased (p<0.05). A moderate negative correlation was observed between functional parameters and quantification of image findings.
  
  
  CONCLUSIONS
  Functional and radiologic alterations suggestive of ILD in long-term MCTD patients are prevalent, mild, and progressed slightly over time. The most sensitive parameters for detecting subtle progression of ILD in MCTD patients are trends in DLCO, quantification of lower-lobes disease by HRCT (lower-lobes %ILD-HRCT score), and qualitative analysis of HRCT imaging.
authors.affiliation: Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Leticia
authors.initials: L
authors.lastname: Kawano-Dourado
authors.affiliation: Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Bruno G
authors.initials: BG
authors.lastname: Baldi
authors.affiliation: Radiology Institute, University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Fernando U
authors.initials: FU
authors.lastname: Kay
authors.affiliation: Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Olivia M
authors.initials: OM
authors.lastname: Dias
authors.affiliation: Radiology Institute, University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Thais E H
authors.initials: TE
authors.lastname: Gripp
authors.affiliation: Pulmonary Division, Hospital do Servidor Publico Estadual (HSPE), Sao Paulo, Brazil.
authors.forename: Paula S
authors.initials: PS
authors.lastname: Gomes
authors.affiliation: Rheumatology Division, Hospital das Clinicas, University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Fuller
authors.affiliation: Rheumatology Division, Hospital das Clinicas, University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Maria T C
authors.initials: MT
authors.lastname: Caleiro
authors.affiliation: Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Ronaldo A
authors.initials: RA
authors.lastname: Kairalla
authors.affiliation: Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School, Sao Paulo, Brazil.
authors.forename: Carlos R R
authors.initials: CR
authors.lastname: Carvalho
mesh_terms: D000328:Adult; D018450:Disease Progression; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000175:diagnosis; D011653:Pulmonary Diffusing Capacity; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity