KL-6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis

. Fathi M, Barbasso Helmers S, Lundberg IE (Karolinska University Hospital, Stockholm; Karolinska Institutet, Karolinska University Hospital, Stockholm; and Institute of Environmental Medicine, Karolinska Institutet, Stockholm; Sweden). KL-6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis. J Intern Med 2012; 271: 589-597. Objectives. To investigate whether Caucasian patients with polymyositis (PM) or dermatomyositis (DM) and interstitial lung disease (ILD) have elevated serum levels of KL-6 compared with patients without ILD and whether KL-6 could be used as a marker for ILD activity and treatment efficacy of ILD in PM/DM. Design and methods. Thirty patients with PM/DM (seven with ILD) and 17 age- and sex-matched healthy controls were included in a retrospective, cross-sectional analysis. Twelve patients were followed for longitudinal evaluation. ILD was defined as restrictive lung function impairment with radiographic signs of ILD. Serum KL-6 levels were measured using a sandwich enzyme immunoassay kit. Groups were compared by MannWhitney U-test. Results. PM/DM patients with ILD had significantly higher median serum KL-6 levels compared with those without ILD: 995 (range 5332318) versus 322 (range 1321225) U mL-1 (P = 0.0002). Median serum levels of healthy controls were 225 (range 136519) U mL-1. Serum levels of KL-6 were inversely correlated with percentages of forced expiratory volume in 1 s (FEV1), vital capacity (VC), total lung capacity (TLC), forced VC, diffusing capacity of carbon monoxide (DLco), maximal voluntary ventilation at 40 breaths min-1 and residual volume (RV). Changes in KL-6 levels showed a significant inverse correlation with changes in percentage FEV1, TLC, DLco and RV. At a cut-off level of 549 U mL-1 (mean +/- 2.5 SD for controls), the sensitivity and specificity for diagnosis of ILD were 83% and 100%, respectively. Conclusion. The level of serum KL-6 may serve as measure of ILD in patients with PM/DM and is a promising biomarker for use in clinical practice to assess clinical response to treatment.