Serum KL-6 as a marker for bronchiolitis obliterans syndrome after lung transplantation.

Bronchiolitis obliterans (BO) is the pathologic manifestation of chronic allograft rejection in lung transplant recipients and specific diagnosis requires invasive tests. BO causes progressive obstruction of the small airways. The term bronchiolitis obliterans syndrome (BOS) is a clinical surrogate for the histopathologic diagnosis of BO and is measured by lung function testing. KL-6 is a glycoprotein expressed on pulmonary epithelial cells and it is present in the serum of normal individuals in small amounts. Serum KL-6 has been shown to be a useful marker of disease activity in interstitial lung diseases. We demonstrated that serum levels of KL-6 are elevated in lung transplant recipients with BOS when compared with those without BOS and healthy controls. Our results indicate that serum KL-6 measurement has the potential to serve as a noninvasive diagnostic test for the detection of BO in lung transplant recipients.

Serum KL-6 and surfactant proteins A and D in pediatric interstitial lung disease.

OBJECTIVE: To determine if serum KL-6, surfactant protein A (SP-A), and surfactant protein D (SP-D) levels are elevated in pediatric interstitial lung disease (ILD) and associated with pulmonary function and disease severity score. METHODS: Serum KL-6, SP-A, and SP-D levels were measured by enzyme-linked immunosorbent assay in 10 children with ILD and in 10 healthy volunteers. In the ILD group, FEV1 percentage of predicted, FVC percentage of predicted, and ILD disease severity score were measured and correlated with serum KL-6, SP-A, and SP-D levels. RESULTS: For the ILD and control groups, respectively, mean serum KL-6 was 4,523 U/mL and 206 U/mL (p = 0.007), mean serum SP-A was 133 ng/mL and 21 ng/mL (p = 0.003), and mean serum SP-D was 304 ng/mL and 75 ng/mL (p = 0.004). There was an inverse relationship between SP-A and FVC (p = 0.05), and between SP-D and FEV1 (p = 0.05). There was a direct relationship between SP-D and ILD score (p = 0.05). CONCLUSIONS: Serum KL-6, SP-D and SP-D levels are elevated in children with ILD. SP-A and SP-D levels appear to correlate with some measures of disease severity.