Objective To analyze the clinical features of MDA5 antibody positive dermatomyositis(MDA5-DM)and to provide evidence for early diagnosis and treatment.Methods From March 2019 to June 2021,272 patients with anti-MDA5-DM from the Nanjing Medical University myositis-associated interstitial lung disease cohort were enrolled,with 76 patients with anti-synthetase syndrome(ASS)as the control group.The clinical characteristics and the occurrence of interstitial lung disease were analyzed.T-test was used for normally distributed and variance-homogeneous independent samples,Mann-Whitney U test for non-normally distributed data,and chi-square test or Fisher's exact test for dichotomous variables.Results Among the 272 anti-MDA5-DM patients,88.6％(241/272)developed interstitial lung disease(ILD),and 33.8％(92/272)developed rapidly progressive ILD(RP-ILD).The six-month all-cause mortality rate of anti-MDA5-DM patients was 16.9％(46/272),and it was as high as 47.8％(44/92)for those with RP-ILD.Compared with ASS patients,anti-MDA5-DM patients had a significantly higher proportion of males,arthritis,Gottron's sign,heliotrope rash,V-sign,periungual erythema,and skin ulcers(P＜0.05).The levels of ALT,AST,and ferritin were significantly increased(P＜0.05).Compared with non-RP-ILD patients,RP-ILD patients had a significantly higher proportion of males[35.9％(33/92)vs.23.3％(42/180),x2=4.79,P=0.029],higher levels of LDH[387(276,547)U/L vs.310(245,400)U/L,Z=-3.67,P＜0.001],ESR[45.5(29.25,63.25)mm/1 h vs.31.2(20,51)mm/1 h,Z=-3.71,P＜0.001],CRP[10.9(4.1,25.2)mg/L vs.4.54(2.58,9.08)mg/L,Z=-4.97,P＜0.001],ferritin[1 340(650,2 000)ng/ml vs.556(203,1 186)ng/ml,Z=-4.40,P＜0.001],and a higher proportion of anti-Ro52 antibody and anti-MDA5 antibody co-positivity[87.0％(80/92)vs.52.2％(94/1 80),x2=31.87,P＜0.001].Conclusion Anti-MDA5-DM patients are prone to develop RP-ILD and have poor prognosis.
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