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.

Study design: 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.

Conflict of interest statement

The authors declare no conflicts of interest.

Copyright © 2016 Elsevier Inc. All rights reserved.

Figures

Figure 1

Log of IL-6 serum level versus forced vital capacity (n = 49). By the use of linear regression, the log value of IL-6 was associated with lower FVC% such that a 10-fold increase in serum IL-6 level was associated with a 14.5% reduction in FVC% (P = .012; n = 49).

Figure 2

Distribution of subjects by quadrants based on normal log values of serum IL-6 and IL-8 levels (n = 55).