Abstract: 【Abstract】Objective To investigate the efficacy and safety of hemoperfusion combined with drugs for the treatment of anti-MDA5 positive dermatomyositis complicated with rapidly progressive interstitial lung disease (RP- ILD). Methods We retrospectively studied 12 anti-MDA5 positive dermatomyositis patients complicated with RP-ILD hospitalized in the period from March 2016 to November 2018. They were treated with HA-280 resin hemoperfusion apparatus for hemoperfusion and the drugs of methylprednisolone, tacrolimus and/or cyclophosphamide. Their peripheral blood lymphocyte count, serum IL-6, IL-10, CRP, Krebs Von den Lungen-6 (KL-6) and ferritin, arterial partial pressure of O2 and CO2, and lung high resolution computed
tomography (HRCT) score were recruited before and after the treatment. ANOVA was used to analyze the data before the treatment and after the treatment for 2 and 4 weeks. LSD-t test was used for the comparison of two groups of data. Results After the treatment for 4 weeks, serum IL-6、IL-10 、KL-6 、ferritin and CRP decreased significantly (F=6.593, 9.029, 7.872, 8.760, 20.490；P=0.005, 0.001, 0.002, 0.001, ＜0.001). Arterial O2 partial pressure、arterial CO2 partial pressure、peripheral blood lymphocyte count improved significantly (F=3.580, 3.467, 16.810；P=0.042,0.046,＜0.001), as compared to those before the treatment. HRCT score (F=2.073, P=0.145) also improved but without statistical significance. Conclusion Hemoperfusion combined with methylprednisolone, tacrolimus and/or cyclophosphamide were effective and safe for the treatment of anti-MDA5 positive dermatomyositis complicated with RP-ILD.

Key words: Melanoma differentiation-associated gene 5, Dermatomyositis, Rapidly progressive interstitial lung disease, Hemoperfusion, Treatment