Objective: To determine whether changes in serum KL-6 levels reflect the activity of pulmonary fibrosis (PF) in patients with systemic sclerosis (SSc).
Methods: KL-6 levels were determined by ELISA in 39 SSc patients. In a retrospective longitudinal study, 250 serum samples were analyzed during a follow-up period of 0.3-6.1 years.
Results: KL-6 levels at the first visit were higher in patients with SSc, especially with PF, compared with healthy controls. In the longitudinal study, KL-6 levels in 4 patients with anti-topo I Abs increased rapidly, parallel to the progression of PF. Four patients with inactive PF exhibited elevated, but stable levels of KL-6 during the follow-up. The 31 patients with almost normal KL-6 levels during the follow-up exhibited no deterioration or new onset of PF.
Conclusion: Rapidly increased serum KL-6 levels during disease course were associated with new onset or deterioration of PF.