PT  - JOURNAL ARTICLE
AU  - Jonas Schupp
AU  - Gabriela Riemekasten
AU  - Corina Kollert
AU  - Benedikt Jäger
AU  - Joachim Müller-Quernheim
AU  - Antje Prasse
TI  - CCL18 as marker of disease progression in systemic sclerosis
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - P692
VI  - 40
IP  - Suppl 56
4099  - http://erj.ersjournals.com/content/40/Suppl_56/P692.short
4100  - http://erj.ersjournals.com/content/40/Suppl_56/P692.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - Background:Lung fibrosis is the most common cause of death in systemic sclerosis. CCL18 is associated with poor prognosis and disease deterioration in fibrotic lung diseases. The prognostic relevance in patients with interstitial lung disease in systemic sclerosis was shown by Tiev et al. (Tiev KP, Eur Respir J 2011 38:1355-1360). Objectives:To evaluate the role of CCL18 in patients with or without interstitial lung disease in systemic sclerosis. Methods:We measured the chemokine CCL18 in sera of 126 patients with progressive systemic sclerosis by ELISA, as well as lung function testing at baseline and every 6 months during follow-up. Pulmonary fibrosis was detected by HR-CT. We computed ROC- and Kaplan-Meier-curves and Cox proportional hazards models to analyze the influence of CCL18 on time to disease progression, defined as decline of the predicted FVC -10 % or death. Results:Patients with serum CCL18 concentrations above 140 ng/ml suffered from a significantly higher chance of disease progression (p<0.0001) within one year. The hazard ratio to suffer from a disease progression was 8.9 in the univariat Cox hazard model and 8.7 in the multivariate Cox hazard model (after adjusting for age, gender and baseline FVC). In the subgroup of patients without pulmonary fibrosis at baseline the hazard ratio was even 21.1 (p<0.0001). Conclusion:CCL18 predicts disease progression of the lung involvement in patients with systemic sclerosis, independent of age, gender or baseline FVC, especially in patients without any evidence of lung fibrosis. CCL18 in sera might therefore be a tool to identify patients who will suffer from lung function deteroriation in the future and to guide therapeutical interventions in these patients.