title: Serum Interleukin-6 Levels and Pulmonary Function in Ataxia-Telangiectasia.
abstract: OBJECTIVE
  To evaluate the potential link between systemic inflammation and impaired lung function in people with ataxia-telangiectasia (A-T), we hypothesized that serum levels of interleukin (IL)-6, a proinflammatory cytokine, would correlate inversely with lung function in subjects with A-T.
  
  
  METHODS
  Consecutive subjects with A-T were recruited from the Johns Hopkins Outpatient A-T Clinical Center. Serum levels of IL-6 and 8 were measured by enzyme-linked immunosorbent assay. Spirometry was performed in subjects ≥ 6 years of age on the same day that serum was obtained for measurements of cytokines.
  
  
  RESULTS
  Approximately 80% of subjects had elevated serum IL-6 levels (> 1.0 pg/mL). No association was found between elevated IL-6 and age. Elevated IL-8 levels were found in 23.6% of subjects, and all subjects with elevated IL-8 levels had elevated IL-6 levels. Subjects with elevated IL-6 levels (mean: 6.14 ± 7.47 pg/mL) had significantly lower mean percent forced vital capacity (FVC%, 50.5% ± 17.8%) compared with subjects with normal serum IL-6 levels (FVC% of 66.2 ± 16.1, P = .018). Greater IL-6 levels were associated with lower FVC% even after adjustment for receiving gamma globulin therapy (P = .024) and supplemental nutrition (P = .055).
  
  
  CONCLUSIONS
  An association was found between elevated serum IL-6 levels and lower lung function in subjects with A-T. In addition, subjects with both elevated IL-6 and IL-8 had the lowest mean lung function. These findings indicate that markers for systemic inflammation may be useful in identifying individuals with A-T at increased risk for lower lung function and may help in assessing response to therapy.
authors: Division of Pediatric Pulmonology, The Johns Hopkins Medical Institutions, Baltimore, MD. Electronic address: smcgrath@jhmi.edu.
authors: Sharon A
authors: SA
authors: McGrath-Morrow
authors: Division of Pediatric Pulmonology, The Johns Hopkins Medical Institutions, Baltimore, MD.
authors: Joseph M
authors: JM
authors: Collaco
authors: Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD.
authors: Barbara
authors: B
authors: Detrick
authors: Division of Pediatric Allergy and Immunology, The Johns Hopkins Medical Institutions, Baltimore, MD.
authors: Howard M
authors: HM
authors: Lederman
mesh_terms: D000293:Adolescent; D001260:Ataxia Telangiectasia / Q000097:blood* / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007249:Inflammation; D015850:Interleukin-6 / Q000097:blood*; D016209:Interleukin-8 / Q000097:blood; D008297:Male; D010641:Phenotype; D012129:Respiratory Function Tests; D013147:Spirometry; D055815:Young Adult
chemical_list: C508606:CXCL8 protein, human; C508600:IL6 protein, human; D015850:Interleukin-6; D016209:Interleukin-8