title: Pulmonary function and high-resolution CT findings five years after disease onset in patients with Wegener's granulomatosis.
abstract: OBJECTIVE
  Although pulmonary involvement is common in Wegener's granulomatosis (WG), little is known about the pulmonary outcome. We evaluated the relationship between clinical disease characteristics and pulmonary function and high-resolution computed tomography (HRCT) findings after disease duration of 5 years.
  
  
  METHODS
  A pulmonary function test (PFT) and pulmonary HRCT were performed in 41 patients from a population-based register of WG. Clinical predictors for abnormal PFT and HRCT were tested by logistic regression.
  
  
  RESULTS
  Previous WG-related lung involvement (PLI) had occurred in 80% of patients, but only 24% of patients still reported pulmonary symptoms at the research visit. One-third of patients had abnormal PFT findings, with reduced alveolar diffusion by KCO (transfer coefficient) being most common (24%). The number of PLI episodes was associated with reduced KCO and reduced FEV1% (forced expiratory volume in 1 s as a percentage of forced vital capacity) (overall presence 10%). Reduced KCO was also associated with disease duration. Reduced total lung capacity (TLC) (overall presence 8%) was only related to prior WG-related lung nodules. Pulmonary HRCT was abnormal in 80%, but with more severe abnormalities in only 30%. Pleural thickening and parenchymal bands were associated with PLI. None of the treatment variables was associated with the PFT or HRCT findings.
  
  
  CONCLUSIONS
  Five years after disease onset a quarter of the WG patients reported pulmonary symptoms, had severe abnormalities on HRCT, and abnormal PFT. The correlation between these abnormalities was poor, but the number of pulmonary involvements was a risk factor for reduced gas diffusion, obstructive lung disease, parenchymal bands, and pleural thickening. Treatment variables had no discernible negative pulmonary effects.
authors.affiliation: Department of Rheumatology, University Hospital Northern Norway, Tromsø, Norway. wenche.koldingsnes@unn.no
authors.forename: W
authors.initials: W
authors.lastname: Koldingsnes
authors.forename: E A
authors.initials: EA
authors.lastname: Jacobsen
authors.forename: T
authors.initials: T
authors.lastname: Sildnes
authors.forename: A
authors.initials: A
authors.lastname: Hjalmarsen
authors.forename: H C
authors.initials: HC
authors.lastname: Nossent
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D002648:Child; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology*; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009664:Norway / Q000453:epidemiology; D011159:Population Surveillance; D012042:Registries; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D014109:Total Lung Capacity / Q000502:physiology