Source: Annual Congress 2002 - Interstitial lung disorders: markers of disease activity and severity
Disease area: Interstitial lung diseases

Abstract

CC16, KL-6 and SP-D are lung epithelium-specific proteins, and potential markers of interstitial lung diseases. The diagnostic value of serum CC16, KL-6 and SP-D to differentiate patients with active sarcoidosis from healthy controls, and their ability to reflect pulmonary disease severity in sarcoidosis has never been compared.
We studied 79 patients with histologically confirmed active sarcoidosis and 38 controls. In all sarcoid patients serum CC16, KL-6 and SP-D concentrations were measured, and pulmonary function tests and chest radiographs were available for evaluation.
All three markers were significantly co-correlated (CC16 and KL-6: r=0.67,p<0.0001; CC16 and SP-D:r=0.63,p<0.0001; KL-6 and SP-D: r=0.63,p<0.0001).
A significant difference was found between CC16, KL-6 and SP-D levels in sarcoid patients and controls (p<0.0001). ROC curve analysis revealed largest area under the curve for KL-6, indicating highest sensitivity and specificity.
Comparing CC16, KL-6 and SP-D levels with clinical severity parameters revealed significant higher levels of CC16 and KL-6 in patients with parenchymal infiltration (stage II and III) compared to patients without parenchymal infiltration (stage I) (CC16: p=0.009; KL-6: p<0.0001). Furthermore, KL-6 was significantly correlated with both DLCO (r=-0.40,p<0.0001) and IVC (r=-0.27,p=0.017), and CC16 with DLCO (r=-0.32,p=0.005).
In conclusion, CC16 and KL-6 appear to be best markers for active pulmonary sarcoidosis, and reflect disease severity. These findings are compatible with the assumption that CC16 relates to increased lung permeability and KL-6 to pneumocyte type II hyperplasia.
Supported by a grant from Astra Zeneca

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R. Janssen, H. Sato, J. C. Grutters, A. Bernard, H. Van Velzen-Blad, K. I. Welsh, R. M. du Bois, J. M. M. van den Bosch (Nieuwegein, The Netherlands; London, United Kingdom; Brussels, Belgium). Comparative study of CC16, KL-6 and SP-D as serum markers in sarcoid patients. Eur Respir J 2002; 20: Suppl. 38, 1117

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