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Objective To investigate the clinical features and prognosis of melanoma differentiation associated protein-5(MDA5)antibody and anti-Ro-52 antibody in double-positive dermatomyositis.Methods Forty-seven dermatomyositis patients with anti-MDA5 antibody positive admitted to the Second Affiliated Hospital of Air Force Military Medical University,Tangdu Hospital from August 2018 to July 2022 were collected.According to whether anti-Ro-52 antibody was positive,they were divided into MDA5 + Ro-52 pos-itive group(n =23)and MDA5 + Ro-52 negative group(n =24).The clinical data of the two groups were retrospectively analyzed,and the differences in the clinical characteristics,laboratory indicators,incidence of rapidly progressive interstitial lung disease and mortality between the two groups were compared.Results Compared between the two groups,the incidence of Gotton rash and hoarseness in the MDA5 + Ro-52 positive group was higher than that in the MDA5 + Ro-52 negative group,and the difference was statistically significant(P<0.05).There were no significant difference in the incidence of skin ulcers,periapillary erythema,positive rash,cape sign,fever,joint pain and sore throat(P>0.05).Lymphocyte count[0.65(0.50,0.81)×109/L vs 1.18(0.91,1.63)×109/L,z =-3.821,P =0.001]and serum albumin[33.40(29.40,35.67)g/L vs 37.25(32.65,40.27)g/L,z =-3.325,P =0.001],oxygen partial pressure[66.60(58.60,86.80)mmHg vs 88.60(75.67,95.72)mmHg,z =-2.373,P = 0.018],blood oxygen saturation[90.40%(89.00%,95.00%)vs 94.90%(90.50%,97.73%),z =-2.353,P = 0.019]in MDA5 + Ro-52 positive group were lower than those in MDA5 + Ro-52 negative group,and the difference were statistically significant(P<0.05).Erythrocyte sedimenta-tion rate[41.00(30.00,62.50)mm/h vs 28.50(21.50,48.75)mm/h,z =2.161,P =0.031]and serum lactate dehydrogenase lev-els[426.00(335.50,605.50)U/L vs 260.00(217.50,373.25)U/L,z =3.313,P =0.011],serum ferritin level[1210.00(465.50,2749.00)μg/L vs 366.00(150.25,629.25)μg/L,z =2.856,P =0.004],the incidence of rapidly progressive interstitial lung disease(73.91%vs 25.00%,χ2 =11.245,P =0.001)and mortality(43.47%vs8.33%,χ2 =7.630,P =0.006)in MDA5 + Ro-52posi-tive group were higher than those in anti-MDA5 + Ro-52 negative group,and the differences were statistically significant(P<0.05).Conclusion Dermatomyositis patients with double-positive anti-MDA5 antibody and anti-Ro-52 antibody are more likely to have increased serum serum lactate dehydrogenase and serum ferritin,decreased serum albumin and peripheral blood lymphocyte count,and more likely to be complicated with rapidly progressive interstitial lung disease and hypoxemia.The prognosis is poor and the mortality is high,which should be paid attention to by clinicians.
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OBJECTIVE@#To investigate the value of serum and bronchoalveolar lavage fluid (BALF) chitinase-3-like-1 protein (YKL-40) in the diagnosis of anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis (DM) patients complicated with serious pulmonary injury, including rapidly progressive interstitial lung disease (RP-ILD) and pulmonary infection.@*METHODS@#Anti-MDA5 antibodies positive patients with DM who were hospitalized in the Department of Rheumatology of China-Japan Friendship Hospital from 2013 to 2018 were involved in this study. Demographic information, clinical, laboratory and imaging data were retrospectively collected. ELISA was used to detect the serum and BALF levels of YKL-40. The receiver operating characteristic (ROC) curve was drawn, and the area under ROC curve (AUC) was used to evaluate the diagnostic value of serum YKL-40 for pulmonary injury.Interstitial lung disease (ILD) was confirmed by chest high-resolution CT (HRCT). RP-ILD was defined as progressive respiratory symptoms such as dyspnea and hypoxemia within 3 months, and/or deterioration of interstitial changes or appearace of new pulmonary interstitial lesions on chest HRCT. Pulmonary infection was considered as positive pathogens detected in qualified sputum, blood, bronchoalveolar lavage fluid or lung biopsy specimens.@*RESULTS@#A total of 168 anti-MDA5-positive DM patients including 108 females and 60 males were enrolled in the study. Of these patients, 154 had ILD, and 66(39.3%) of them presented RP-ILD. Seventy patients with pulmonary infection were confirmed by etiology. In the patients with RP-ILD, 39 (59.1%) of them were complicated with pulmonary infection. While only 31 cases(30.4%) had pulmonary infection in the non-RP-ILD patients. The incidence of pulmonary infection in the patients with RP-ILD was significantly higher than that of those with non-RP-ILD (P < 0.001). The serum YKL-40 levels in the RP-ILD patients with pulmonary infection were the highest compared with RP-ILD without pulmonary infection, non-RP-ILD with pulmonary infection and non-RP-ILD without pulmonary infection groups among all the patients [83 (42-142) vs. 42 (21-91) vs. 43 (24-79) vs. 38 (22-69), P < 0.01].The sensitivity, specificity and AUC of serum YKL-40 in the diagnosis of RP-ILD complicated with pulmonary infection were 75%, 67%, and 0.72, respectively. The AUC of diagnosed of anti-MDA5 positive DM patients complicated with RP-ILD and pulmonary infection was higher than that of patients complicated with only RP-ILD and only pulmonary infection (0.72 vs. 0.54 and 0.55, Z=2.10 and 2.11, P < 0.05).@*CONCLUSION@#The prognosis of anti-MDA5-positive DM patients with RP-ILD and pulmonary infection were poor. Serum YKL-40 level can be used as a helpful tool for the diagnosis of coexistence of these conditions in the patients.