[Clinical characteristics and prognostic factors of patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis associated interstitial lung disease].

Objective: To analyze the clinical characteristics and prognostic factors of patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive dermatomyositis associated interstitial lung disease (DM-ILD). Methods: The patients with MDA5 + DM-ILD who were admitted to Department of Respiratory Medicine, Nanjing Drum Tower Hospital from January 2017 to March 2021 were enrolled. The clinical data and survival information were analyzed retrospectively. Patients were divided into survival group or death group, and rapid progressive ILD (RP-ILD) group or non-rapid progressive ILD group, according to their survival status and clinical progression. Results: A total of 105 patients with anti-MDA5 + DM-ILD (median age of onset 54 years) were enrolled, 58% being female (61 cases). The main sub-type of dermatomyositis was amyopathic dermatomyositis ( n =74, 70%), followed by dermatomyositis ( n =31, 30%). The main extrapulmonary manifestations were skin lesions ( n =60, 57.1%), muscle manifestations( n =20, 19%) and arthralgia / arthritis ( n =20, 19%). 15.4% of the patients had positive ANA (antibody titer≥1∶320), and 61.9% of the patients had anti-RO-52 kDa antibody. A total of 66 patients (62.8%) developed RP-ILD, and 58 patients (56.3%) died. Lower oxygenation index ( OR =0.974, 95% CI :0.954-0.994, P= 0.012) and no joint pain ( OR =0.032, 95% CI : 0.002-0.663 P= 0.026) were independent risk factors for RP-ILD. Cox regression analysis showed that RP-ILD ( HR =3.194, 95% CI :1.025-9.954, P= 0.045), older than 53 years ( HR =3.450, 95% CI : 1.388-8.577, P= 0.008), ferritin level more than 1 330.5 ng/ml ( HR =3.032, 95% CI 1.208-7.610, P= 0.018) and C-reactive protein (CRP) above 16.95 mg/L ( HR =2.794, 95% CI :1.102-7.084, P =0.030) were independent predictors of mortality. Conclusions: The clinical manifestations of patients with anti-MDA5+DM-ILD presenting to the respiratory department were heterogeneous, with most being amyopathic dermatomyositis, and both the incidence of RP-ILD and the risk of death were high. Even in the absence of associated rash, joint, or muscle manifestations, anti-MDA5 antibody screening should be considered in patients with rapidly progressive ILD who were negative on baseline autoantibody screening but positive for anti-RO52kDa antibody.