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Thorax 2003;58:613-617

© 2003 BMJ Publishing Group & British Thoracic Society

Expression of S100A12 (EN-RAGE) in cystic fibrosis

D Foell1,2, S Seeliger1,2, T Vogl2, H-G Koch1, H

Maschek3, E Harms1, C Sorg2 and J Roth1,2

1 Department of Paediatrics, University of Muenster,

Germany

2 Institute of Experimental Dermatology, University of

Muenster, Germany

3 Institute of Pathology, Hannover, Germany

Correspondence to:

Dr D Foell, Department of Paediatrics, University

Hospital Muenster, Albert-Schweitzer-Strasse 33,

D-48149 Muenster, Germany;

dfoell@...

ABSTRACT

Background: Chronic airway inflammation and recurrent

infections are a core phenomenon in cystic fibrosis

(CF). Diagnosing acute infectious exacerbations is

difficult in the presence of chronic inflammatory

processes. S100A12 exhibits proinflammatory functions

via interaction with the multiligand receptor for

advanced glycation end products. Blocking this

interaction inhibits inflammatory processes in mice.

Methods: The expression of S100A12 in lung specimens

of patients with end stage lung disease of CF was

investigated, and S100A12 levels in the serum of

patients with acute infectious exacerbations of CF

were measured.

Results: Immunohistochemical studies of CF lung biopsy

specimens revealed a significant expression of S100A12

by infiltrating neutrophils. High S100A12 levels were

found in the sputum of patients with CF, and serum

levels of S100A12 during acute infectious

exacerbations were significantly increased compared

with healthy controls (median 225 ng/ml v 46 ng/ml).

After treatment with intravenous antibiotics the mean

S100A12 level decreased significantly. There was also

a significant difference between S100A12 levels in

patients with acute infectious exacerbations and 18

outpatients without exacerbations (median 225 ng/ml v

105 ng/ml).

Conclusions: S100A12 is extensively expressed at local

sites of inflammation in CF. It is a serum marker for

acute infectious exacerbations. High local expression

of S100A12 suggests that this protein has a

proinflammatory role during airway inflammation and

may serve as a novel target for anti-inflammatory

treatments.

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