title: Inflammatory markers of lung disease in adult patients with cystic fibrosis.
abstract: BACKGROUND
  Progressive pulmonary disease associated with chronic bacterial infection and inflammation is the major cause of morbidity and mortality in cystic fibrosis (CF) patients. Identifying markers of inflammation that correlate with lung injury may be useful in monitoring disease progression and response to therapy. We hypothesized that levels of serum biomarkers would correlate with clinical course of CF as defined by pulmonary function testing (FEV1).
  
  
  OBJECTIVE
  To determine whether biomarkers of systemic inflammation correlate with lung function in adults with CF.
  
  
  METHODS
  Retrospective cross-sectional analysis of 63 individuals > or = 30 years of age diagnosed with CF in childhood and followed at Children's Hospital, Boston. We collected data on demographics, CFTR genotype, percent predicted forced expiratory volume in 1 sec (FEV1), C-reactive protein (CRP), serum IgE nd IgG, alpha1-antitrypsin, total white blood cell and neutrophil counts, and percent neutrophils. We used univariate analyses and multivariate linear regression modeling to examine whether markers of systemic inflammation varied with FEV1 (% predicted).
  
  
  RESULTS
  In two-covariate models including CRP and one other marker, CRP (P < 0.001) and IgG (P = 0.02) were significantly associated with FEV1 (% predicted). In the CRP and IgG model, percent predicted FEV1 decreased by 4.91% (P < 0.0001) for each twofold increase in CRP and by 1.56% (P = 0.02) for each 100 mg/dl increase in IgG. Results were unchanged by adjustment for number of DF 508 CFTR alleles. There was no association between any other marker and FEV1 (% predicted) after adjusting for CRP.
  
  
  CONCLUSIONS
  Severity of lung disease in long surviving adult CF patients is correlated with CRP and IgG levels. Our findings relating CRP and IgG levels and lung function provide a foundation for subsequent longitudinal studies and consideration of novel disease mechanisms and outcome measurements.
authors.affiliation: Division of Pulmonary Medicine, Children's Hospital Boston, Boston, Massachusetts, USA. Hara.Levy@childrens.harvard.edu
authors.forename: Hara
authors.initials: H
authors.lastname: Levy
authors.forename: Leslie A
authors.initials: LA
authors.lastname: Kalish
authors.forename: Ian
authors.initials: I
authors.lastname: Huntington
authors.forename: Nathaniel
authors.initials: N
authors.lastname: Weller
authors.forename: Craig
authors.initials: C
authors.lastname: Gerard
authors.forename: Edwin K
authors.initials: EK
authors.lastname: Silverman
authors.forename: Juan C
authors.initials: JC
authors.lastname: Celedón
authors.forename: Gerald B
authors.initials: GB
authors.lastname: Pier
authors.forename: Scott T
authors.initials: ST
authors.lastname: Weiss
mesh_terms: D000328:Adult; D002097:C-Reactive Protein / Q000032:analysis; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000097:blood* / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007249:Inflammation / Q000150:complications; D008171:Lung Diseases / Q000097:blood* / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D008297:Male; D012189:Retrospective Studies
chemical_list: D007074:Immunoglobulin G; D002097:C-Reactive Protein