KL-6 is a potential marker for interstitial lung disease associated with juvenile dermatomyositis.

Serum levels of KL-6 were examined in 8 cases of juvenile dermatomyositis: 3 with interstitial lung disease (ILD) and 5 without ILD. The KL-6 levels were elevated in the ILD cases and correlated with the degree of computed tomography findings. The measurement of serum KL-6 levels is useful for evaluating juvenile dermatomyositis-associated ILD.