Objective To explore the association of serum cytokine levels with disease activity in patients with dermatomyositis (DM) and clinically amyopathic dermatomyositis (CADM) , especially their association with skin lesions and interstitial lung disease (ILD) . Methods Enzyme-linked immunosorbent assay (ELISA) and cytometric beads array (CBA) were performed to detect the serum levels of interleukin (IL) -2, IL-4, IL-6, IL-10, IL-17A, IL-18, tumor necrosis factor (TNF) and interferon (IFN) -γ in 40 patients with DM or CADM, as well as in 16 health checkup examinees (healthy control group) . Then, the association of serum cytokine levels with skin lesions, inflammatory biomarkers and severity of ILD was analyzed. Results The patients with DM/CADM showed significantly higher serum levels of IL-6 (37.8 ± 45.8 pg/ml) , IL-10 (16.1 ± 7.2 pg/ml) and IL-18 (492.0 ± 193.1 pg/ml) compared with the healthy controls (12.0 ± 2.7 pg/ml, 7.7 ± 1.4 pg/ml, 191.1 ± 39.2 pg/ml, respectively, all P < 0.001) , and there were no significant differences in the serum levels of the other 5 cytokines between the above 2 groups. The serum level of IL-6 was significantly higher in patients with elevated erythrocyte sedimentation rate (ESR) than in those with normal ESR (49.7 ± 46.8 pg/ml vs. 29.1 ± 45.4 pg/ml, P= 0.008) . The patients with raised C-reactive protein (CRP) levels showed significantly higher serum levels of IL-6 (68.7 ± 59.7 pg/ml) and IL-18 (635.1 ± 232.8 pg/ml) compared with those with normal CRP levels (IL-6: 30.6 ± 40.3 pg/ml, P= 0.013; IL-18: 440.2 ± 164.7 pg/ml, P= 0.020) . Moreover, the patients with elevated levels of lactate dehydrogenase (LDH) showed significantly higher serum levels of IL-10 (18.4 ± 6.9 pg/ml) , IL-17A (19.6 ± 6.7 pg/ml) and IL-18 (529.4 ± 197.2 pg/ml) compared with those with normal LDH levels (IL-10: 10.7 ± 4.8 pg/ml, P < 0.001; IL-17A: 11.4 ± 6.6 pg/ml, P= 0.001; IL-18: 404.9 ± 158.0 pg/ml, P= 0.037) . No significant difference in the cytokine levels was observed between the patients with elevated creatine kinase (CK) levels and those with normal CK levels. The patients with Gottron′s papules/sign showed significantly higher serum levels of IL-18 (513.7 ± 187.2 pg/ml) compared with those without Gottron′s papules/sign (297.1 ± 140.4 pg/ml, P < 0.05) . The serum levels of IL-10 and IL-18 were significantly higher in the patients with DM/CADM complicated by ILD (18.0 ± 6.7 pg/ml, 552.3 ± 192.8 pg/ml, respectively) than in those without ILD (11.6 ± 6.5 pg/ml, 351.4 ± 101.0 pg/ml, respectively, both P= 0.001) . Conclusion Serum levels of IL-6, IL-10 and IL-18 are highly associated with inflammatory biomarkers, skin lesions and ILD in patients with DM/CADM. Key words: Dermatomyositis; Cytokines; Lung Diseases, Interstitial; Interstitial lung disease; Clinically amyopathic dermatomyositis