title: Quantifying change in pulmonary function as a prognostic marker in systemic sclerosis-related interstitial lung disease.
abstract: OBJECTIVE
  Clinically meaningful change in systemic sclerosis (SSc) related interstitial lung (SSc-ILD) disease is unknown. The aim of this study was to quantify change in pulmonary function as a predictor of outcome in SSc-ILD.
  
  
  METHODS
  All patients had SSc-ILD defined by HRCT chest. All PFTs during follow-up, including FVC (L), DLCO (ml/min/mmHg) and KCO (DLCO/alveolar volume ratio; DLCO/VA) (ml/min/mmHg/L) were retrieved. The rate of change over the first four years, and percentage change in the first year of follow-up were used in ROC curve analysis to determine the best cut-off points to predict adverse outcome (home oxygen, lung transplantation, or death).
  
  
  RESULTS
  Among 264 patients, there were 49 events (38 deaths, 10 supplemental oxygen, one lung transplant) over a mean (±SD) follow-up of 3.0 (±1.7) years. The rates of decline over time and percentage change over one year in each of FVC, DLCO and KCO were predictive of adverse outcome. Stable PFTs over four years gave the optimal negative predictive values (NPVs) of 88-96%. The best sensitivity-specificity trade-off was a decline in FVC of 10% and in DLCO and KCO of 15% with NPVs of 92-93%.
  
  
  CONCLUSIONS
  The course that SSc-ILD takes is evident within the first 1-4 years of follow up. Patients who have no decline in PFTs over 4 years have better outcomes. A decline within one year in DLCO or KCO of 15% or more is a poor prognostic factor, and identifies patients who should be monitored more closely and considered for therapy.
authors.affiliation: Department of Rheumatology, St. Vincent's Hospital Melbourne, Victoria, Australia, and Department of Rheumatology, Derriford Hospital, Plymouth, Devon, UK.
authors.forename: Owen A
authors.initials: OA
authors.lastname: Moore
authors.affiliation: Rheumatology Unit, Royal Adelaide Hospital; and Discipline of Medicine, University of Adelaide, South Australia.
authors.forename: Susanna M
authors.initials: SM
authors.lastname: Proudman
authors.affiliation: Department of Respiratory Medicine, Austin Hospital Melbourne, Victoria; and Department of Respiratory Medicine, Alfred Hospital Melbourne, Victoria, Australia.
authors.forename: Nicole
authors.initials: N
authors.lastname: Goh
authors.affiliation: Department of Respiratory Medicine, Royal Prince Alfred Hospital Sydney, New South Wales, Australia.
authors.forename: Tamera J
authors.initials: TJ
authors.lastname: Corte
authors.affiliation: Department of Radiology, St. Vincent's Hospital Melbourne, Victoria, Australia.
authors.forename: Hannah
authors.initials: H
authors.lastname: Rouse
authors.affiliation: Department of Radiology, St. Vincent's Hospital Melbourne, Victoria, Australia.
authors.forename: Oliver
authors.initials: O
authors.lastname: Hennessy
authors.affiliation: Department of Rheumatology, St. Vincent's Hospital Melbourne, Victoria, Australia.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Morrisroe
authors.affiliation: Department of Rheumatology, St. Vincent's Hospital Melbourne, Victoria; and Department of Rheumatology, Liverpool Hospital Sydney; and School of Medicine, University of Western Sydney, New South Wales, Australia.
authors.forename: Vivek
authors.initials: V
authors.lastname: Thakkar
authors.affiliation: Department of Rheumatology, Monash Medical Centre Melbourne, Victoria, Australia.
authors.forename: Joanne
authors.initials: J
authors.lastname: Sahhar
authors.affiliation: Department of Rheumatology, Royal Perth Hospital, Western Australia.
authors.forename: Janet
authors.initials: J
authors.lastname: Roddy
authors.affiliation: Department of Rheumatology, Royal Prince Alfred Hospital and Sydney University, New South Wales, Australia.
authors.forename: Peter
authors.initials: P
authors.lastname: Youssef
authors.affiliation: Advanced Lung Disease Unit, Royal Perth Hospital; and The University of Notre Dame, Fremantle, Western Australia.
authors.forename: Eli
authors.initials: E
authors.lastname: Gabbay
authors.affiliation: Rheumatology Research Unit, Department of Medicine, University of Queensland, QLD, Australia.
authors.forename: Peter
authors.initials: P
authors.lastname: Nash
authors.affiliation: The Menzies Research Institute, Tasmania.
authors.forename: Jane
authors.initials: J
authors.lastname: Zochling
authors.affiliation: Department of Rheumatology, St. Vincent's Hospital Melbourne, Victoria, Australia.
authors.forename: Wendy
authors.initials: W
authors.lastname: Stevens
authors.affiliation: Department of Rheumatology, St. Vincent's Hospital Melbourne, Victoria; and The University of Melbourne Department of Medicine, St. Vincent's Hospital Melbourne, Victoria, Australia. m.nikpour@unimelb.edu.au.
authors.forename: Mandana
authors.initials: M
authors.lastname: Nikpour
mesh_terms: D000368:Aged; D019540:Area Under Curve; D001315:Australia; D018450:Disease Progression; D005260:Female; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D011446:Prospective Studies; D011653:Pulmonary Diffusing Capacity; D012372:ROC Curve; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity