title: The significance of elevated tumor markers among patients with idiopathic pulmonary fibrosis before and after lung transplantation.
abstract: BACKGROUND
  Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a 3-year median survival. Lung volume and diffusion capacity at rest are usually used to monitor the clinical course. Because of high mortality, identification of patients at high risk is crucial for treatment strategies such as lung transplantation (LTX). This study was designed to determine if tumor markers could accurately characterize disease severity and survival in patients with IPF.
  
  
  METHODS
  The study population consisted of 61 patients with progressive IPF referred for LTX. Pulmonary function tests, cardiopulmonary exercise test, 6-min walk distance test, and Doppler echocardiogram were assessed at baseline and compared with tumor marker levels. Participants were prospectively followed for at least 25 months to determine the relationship between test parameters and survival. Tumor marker levels were reassessed in patients who underwent LTX. Forty-one age- and sex-matched patients (21 LTX recipients) with COPD served as control subjects.
  
  
  RESULTS
  In the IPF group, nine patients (14.7%) died during follow-up and 20 (32.8%) underwent LTX. Univariate analysis showed correlations between carbohydrate antigen (CA) 125 and FEV(1) % (P = .0001). CA 19-9 yielded the best correlations with exercise parameters and PAP. Significant correlation with survival was noted with CA 15-3 (P = .04) only. All tumor marker levels decreased significantly following LTX, except CA 125. CA 15-3 had the largest decrease (P = .001). Among the COPD group, tumor marker levels before LTX were significantly lower compared with the IPF and did not decrease following LTX. No patient in either group developed malignancy.
  
  
  CONCLUSIONS
  CA 15-3 levels may predict disease severity in IPF. Levels decreased in patients with IPF but not with COPD following LTX and were not associated with malignancy. This preliminary observation suggests that mucin has a role in the pathogenesis of IPF and possibly is a marker for disease activity.
authors.affiliation: Pulmonary Institute, Rabin Medical Center, Beilinson Campus, Petach Tikva, Israel.
authors.forename: Victoria
authors.initials: V
authors.lastname: Rusanov
authors.affiliation: Pulmonary Institute, Rabin Medical Center, Beilinson Campus, Petach Tikva, Israel.
authors.forename: Mordechai R
authors.initials: MR
authors.lastname: Kramer
authors.affiliation: Pulmonary Institute, Rabin Medical Center, Beilinson Campus, Petach Tikva, Israel.
authors.forename: Yael
authors.initials: Y
authors.lastname: Raviv
authors.affiliation: Pulmonary Institute, Rabin Medical Center, Beilinson Campus, Petach Tikva, Israel.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Medalion
authors.affiliation: Pulmonary Department, Meir Medical Center, Kfar Saba, Affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Alexander
authors.initials: A
authors.lastname: Guber
authors.affiliation: Pulmonary Department, Meir Medical Center, Kfar Saba, Affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address: davids3@clalit.org.il.
authors.forename: David
authors.initials: D
authors.lastname: Shitrit
mesh_terms: D014408:Biomarkers, Tumor / Q000097:blood*; D018394:CA-125 Antigen / Q000097:blood; D018395:CA-19-9 Antigen / Q000097:blood; D002272:Carcinoembryonic Antigen / Q000097:blood; D015150:Echocardiography, Doppler; D005080:Exercise Test; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000097:blood / Q000401:mortality / Q000601:surgery*; D016040:Lung Transplantation; D018396:Mucin-1 / Q000097:blood; D011446:Prospective Studies; D029424:Pulmonary Disease, Chronic Obstructive / Q000097:blood / Q000601:surgery; D012129:Respiratory Function Tests; D012720:Severity of Illness Index
chemical_list: D014408:Biomarkers, Tumor; D018394:CA-125 Antigen; D018395:CA-19-9 Antigen; D002272:Carcinoembryonic Antigen; D018396:Mucin-1