Volkmann ER, et al. - Patients who recruited in Scleroderma Lung Study II were included by the experts in order to assess the relationship between Krebs von den Lungen-6 [KL-6] and CC chemokine ligand 2 [CCL-18]) with severity and progression of systemic sclerosis-related interstitial lung disease (SSc-ILD). A correlation of baseline KL-6 and CCL-18 levels with the extent of radiographic fibrosis was observed. On the basis of the course of the forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DL_CO), a greater baseline KL-6 level predicted the progress of ILD, over 1 year. Based on the course of the FVC, DL_CO over 1 year, higher baseline CCL-18 level forecasted progression as well as mortality of ILD. Hence, KL-6 and CCL-18 levels were associated with ILD severity for SSc-ILD. Also, it diminished with immunosuppression. Disease progression was more likely to be encountered by patients with higher baseline KL-6 and CCL-18 levels, despite the treatment. Furthermore, to recognize patients with a progressive ILD phenotype, KL-6 and CCL-18 could be utilized who could profit from a more aggressive initial treatment approach.