| BackgroundDermatomyositis(DM)is a group of autoimmune diseases characterized by rash and muscle inflammation,and interstitial lung disease(ILD)is the most common complication of DM,which seriously affects the prognosis of patients with DM.The progression of ILD varies in different patients,including rapidly progressive ILD(RP-ILD)and chronic ILD(C-ILD).RP-ILD has a very high mortality rate and very poor prognosis.Studies have shown that amyopathic dermatomyositis(ADM),as a special phenotype of DM,was classified as a subtype by the latest classification criteria and was associated with the occurrence of RP-ILD.At present,there is no exact diagnostic criteria,and the diagnosis of RP-ILD mainly relies on clinicians to perform suspicious diagnosis of clinical manifestations and the progress of highresolution CT with close attention.Therefore,it is necessary to analyze the clinical characteristics and risk factors of DM/ADM-RP-ILD,improve the understanding of RP-ILD,and early identify and treat potentially high-risk patients.Objectives1.The aim of this study was to analyze the clinical manifestations,serological parameters and the ch aracteristics of MS As and MAAs in DM/ADM with RP-ILD2.To evaluate the diagnostic value of clinical characteristics and serological markers for RP-ILD in the DM patients with ILD.3.To analyze the survival rate of different groups of DM/ADM patients(RPILD,C-ILD and N-ILD),and explore the effect of different indicators on the survival of DM/ADM-RP-ILD.MethodsThis was a retrospective cohort study,data including demographic characteristics,clinical and laboratory records were collected from the first affiliated hospital of Zhengzhou University.The 2017 EULAR/ACR criteria were used for reclassification diagnosis.According to different phenotypes of ILD,all enrolled patients were divided into three groups:RP-ILD,C-ILD and no ILD(N-ILD).ROC curve was used for diagnostic value analysis;Kaplan-Meier method was used to analyze the cumulative survival rate;Cox proportional hazards regression analysis was u sed to analyze the prognostic risk factors of RP-ILD patients within 6 months.Results1.A total of 317 patients with DM/ADM(161 with DM and 156 with ADM)were included,including 194 patients with DM-ILD(59 with RP-ILD and 135 with C-ILD).Compared with patients with C-ILD,patients with RP-ILD had order age at onset,higher incidence of fever,higher ESR,CRP,ferritin,NLR,CEA,and CA125 levels,and lower incidence of myalgia and DLco levels(P<0.05).2.The positive rate of anti-MDA5 antibody in DM/ADM-RP-ILD patients(88.89%)was significantly higher than that in patients with C-ILD(63.86%)and without ILD(26.67%)(P<0.001).Of the anti-MDA5-related DM/ADM patients,32.99%developed RP-ILD.Anti-TIF1-γ and anti-Mi-2 antibodies were significantly higher in DM/ADM patients without ILD.3.CEA[AUC=0.762,95%CI=(0.681,0.842)]and ferritin[AUC=0.700,95%CI=(0.602,0.798)]were used as indicators for early identification of RP-ILD from the DM/ADM-ILD population.The combination of CEA with ferritin and antiRo-52 antibody,respectively,could improve the diagnosis value.Older age,fever and CEA levels were risk factors for the development of RP-ILD.4.The cumulative survival rate of patients with RP-ILD was significantly lower than that of patients with C-ILD(23.73%vs 89.43%)(P<0.001),while there was no significant difference between patients with C-ILD and N-ILD.AST/ALT(DRR)≥1.42[HR=2.167,95%CI=(1.053,3.614)]was an independent risk factor for death within 6 months in patients with DM/ADM-RP-ILD.Conclusion1.In patients with DM/ADM-RP-ILD,the age of onset,as well as the levels of serum parameters such as ferritin,CEA,and DRR were significantly increased.CEA and ferritin can be used as auxiliary markers to assist clinicians in early identification of DM/ADM-RP-ILD.2.Higher DRR in patients with DM/ADM-RP-ILD were related with high mortality rate within 6 months,and such patients can be treated early with intensive or new treatment to improve their survival rate. |
