Abstract
AIM: A prospective and longitudinal study to investigate the correlations between Krebs von den Lungen-6 (KL-6) serum levels and systemic sclerosis associated with interstitial lung disease (SSc-ILD). METHOD: Blood samples of baseline and the time point at 2 years follow-up intervals were collected for the measurement of serum KL-6 levels. The baseline clinical, laboratory characteristics, and incidence density of newly diagnosed ILD during the follow up were compared between SSc patients with elevated serum KL-6 levels (KL-6 > 500 U/mL) and those with normal KL-6 levels (KL-6 ≤ 500 U/mL) at baseline. Further, we explored the association between serum KL-6 and deterioration of ILD measured by lung function parameters during follow-up of 2 years. RESULTS: Patients with elevated baseline serum KL-6 had a significant tendency to have disappearance of the finger pad. The incidence density of new-onset ILD in SSc patients with elevated baseline serum KL-6 and those with normal baseline serum KL-6 was 1.33% and 0.51%, respectively. Among the mild lung injury group, the incidence density of ILD deterioration in SSc patients with elevated baseline serum KL-6 and those with normal serum KL-6 was 1.2% and 0.74%, respectively. CONCLUSION: Serum KL-6 level correlates with the clinical manifestations of microvascular injury. Baseline elevated serum KL-6 may predict deterioration of lung function of SSc-ILD patients with mild lung injury.