Serum Interleukin-6 Levels and Pulmonary Function in Ataxia-Telangiectasia

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• 作者：Sharon A. McGrath-Morrow ; MD¹ ; ^{smcgrath@jhmi.edu" class="auth_mail" title="E-mail the corresponding author} ; Joseph M. Collaco ; MD ; MPH¹ ; Barbara Detrick ; PhD² ; Howard M. Lederman ; MD ; PhD³
• 关键词：A-T ; Ataxia-telangiectasia ; ATM ; Ataxia-telangiectasia mutated ; DDR ; DNA damage repair ; FEV1% ; Percent Forced expiratory volume in 1 second ; FVC% ; Percent forced vital capacity ; IL ; Interleukin ; IVIG ; Intravenous immunoglobulin
• 刊名：The Journal of Pediatrics
• 出版年：2016
• 出版时间：April 2016
• 年：2016
• 卷：171
• 期：Complete
• 页码：256-261.e1
• 全文大小：428 K

文摘

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.