| Objective:To investigate the clinical characteristics of idiopathic inflammatory myopathy-related interstitial lung disease and its correlation with the expression levels of serum KL-6,Wnt-5a and GDF-15,so as to improve the early diagnosis of inflammatory myopathy-related interstitial pulmonary disease.Methods:In February 2018 to December 2019 were admitted in Ningxia Medical University General Hospital Department of rheumatology and respiratory and critical care medicine diagnosis for patients with idiopathic inflammatory myopathy(IIM)as experimental group 110 cases,all patients were performed chest HRCT,according to the presence of lung interstitial lesions,the severity of lung interstitial lesions,pathological type and type of inflammatory myopathy antibody subgroup analysis the selection in the same period of Other connective tissue lung involvement(Other CTD ILD)29 cases,and Idiopathic Interstitial Pneumonia(IIP)29 cases as control group.Detailed records of clinical data of all included patients(including clinical history,symptoms,signs,laboratory tests,etc.);At the same time,the included patients' serum was retained.The serum levels of KL-6,Wnt-5a and GDF-15 were detected by ELISA.All the included patients' lung imaging data were retained for analysis.The optimal threshold value,sensitivity and specificity of diagnosis of KL-6,Wnt-5a and GDF-15 were obtained by ROC curve analysis.Results:1.General information of the Idiopathic Inflammatory Myopathy group and Idiopathic Inflammatory Myopathy combined with Interstitial Lung Disease group: among the110 IIM patients,42 patients with Idiopathic Inflammatory Myopathy(IIM)accounted for38.1%(42/110),including 12 males(28.5%(12/42)and 30 females(71.5%(30/42)),with a male ratio of 1:2.5 and an average age of(45±16).There were 68 cases(61.8%(68/110)of patients with Idiopathic Inflammatory Myopathy combined with Interstitial Lung Disease(IIM-ILD),including 20 cases(29.4%(20/68)of males and 48 cases(70.6%(48/68)of females and 1:2.4 of males,with an average age of 55±13).The mean age(53±13 vs 45±16)between the iim-ild group and the IIM group was statistically significant(P=0.001).2.Simple Idiopathic Inflammatory Myopathy group merged with idiopathic inflammatory myopathy interstitial pneumonia clinical data set of comparison: between IIM-ILD and pure IIM group,on the clinical manifestations of IIM-ILD group are more likely to show the chest distress,joint pain,fever,Mechanic's hand,IIM group are more likely to show the proximal muscle weakness,difficulty swallowing(P < 0.05).The IIM-ILD group was more likely to have low arterial partial oxygen pressure,high CRP level,low CK level,low AST level and low ALT level(P < 0.05).The IIM-ILD group was more likely to have positive Anti-SSA antibody and myositis specific antibody: positive Anti-Jo-1 antibody and positive Anti-MDA5 antibody(P < 0.05).3.Idiopathic Inflammatory Myopathy with Interstitial Lung Disease group antibody of different types of clinical data comparison: IIM-ILD group is divided into: depending on the type of positive for myositis specificity antibodies against amino acyl RNA synthetase antibodies and interstitial lung disease group(ARS-ILD group),positive for melanoma differentiation related gene antibody five and interstitial lung disease group(MDA5-ILD)and negative for myositis itself specific antibody and interstitial lung disease group(MSA-ILD),between the three groups,MDA5-ILD group had a higher incidence of rash,including:heliotrope rash,V sign,Gottron sign(P <0.05).The highest incidence ofacute/subacute ILD was found in the MDA5-ILD group(P < 0.05).The arterial partial oxygen pressure and CK level were lower in the MDA5-ILD group(P < 0.05).4.Risk factors analysis of inflammatory myopathy with interstitial lung disease:with or without interstitial involvement as the dependent variable,age,fever,joint pain,CRP level,etc,as independent variables,Logistic regression analysis was performed,and the results showed that age,Mechanic's hand,positive Anti-JO-1 and positive Anti-MDA5 antibody were the risk factors for IIM-ILD.5.Pulmonary imaging findings of inflammatory myopathy with interstitial pneumonia: in the iim-ild group,mesh shadow accounted for 69%,ground glass shadow for34%,consolidation shadow for 63.2%,honeycomb shadow for 5.9%,linear shadow for32.3%,tractive bronchiectasis for 23.5%,cystic bubble for 7.4%,and pleural effusion for2.9%.In the image category distribution of interstitial lung disease,NSIP accounted for 32.4%,NSIP combined OP accounted for 23.5%,OP accounted for 7.4%,UIP accounted for 1.4%,possibale UIP accounted for 2.9%,unclassified CT pattern accounted for 32.4%.The interstitial types of the ARS-ILD group were mainly NSIP and NSIP combined OP,accounting for 36.7% and 40%,respectively(P =0.001),and the interstitial types of the MDA5-ILD group were mainly undefined,accounting for 72.2%(P < 0.05).HRCT score of MDA5-ILD group was significantly higher than that of MSA-ILD group [MDA5-ILD vs MSA-ILD,10.5(6.8,13.0)vs 4.0(2.0,6.0),P =0.013].HRCT score of IIP group was significantly higher than that of Other CTD-ILD group [IIP vs Other CTD-ILD,9.0(5.8,12.3)vs 7.0(2.5,9.0),P =0.028].6.Comparative study of serum KL-6,Wnt-5a and GDF-15 in the four groups of inflammatory myopathy group,inflammatory myopathy combined with interstitial disease group,connective tissue disease-related interstitial disease(CTD-ILD group)and idiopathic interstitial lung disease(IIP group):The serum levels of KL-6 in the pure IIM group were significantly lower than the Other three groups [IIM vs IIM-ILD,IIM vs Other CTD-ILD andIIM vs IIP were 284.29(203.3-393.7)vs 726.2(514.5-977.0)U/ml,284.29(203.3-393.7)vs468.1(296.6-977.0)U/ml,and 284.29(203.3-393.7)vs 519.0(393.4-870.6)U/ml,respectively,P <0.05];The serum GDF-15 level in IIM group was significantly higher than that in the Other three groups [IIM vs IIM-ILD,IIM vs Other CTD-ILD and IIM vs IIP were 564.3(391.7-914.3)vs 178.9(119.8-276.5)pg/ml,564.3(391.7-914.3)vs 154.7(95.5-213.2)pg/ml and 564.3(391.7-914.3)vs 252.7(170.6-300.9)pg/ml,,respectively,P<0.05];The serum Wnt-5a level in the IIM group was significantly lower than that in the Other three groups [IIM vs IIM-ILD,IIM vs Other CTD-ILD,IIM vs IIP were 1.3(0.6-2.3)vs1.8(1.1-3.8)ng/ml,1.3(0.6-2.3)vs 1.4(0.8-3.5)ng/ml,1.3(0.6-2.3)vs 1.5(1.1-2.8)ng/ml,,respectively,P <0.05].In order to evaluate the diagnostic value of serum KL-6,GDF-15,and Wnt-5a on IIM-ILD,the receicer operating characteristic curve was analyzed,and the critical level of KL-6 was 431.3u/ml,the sensitivity was 92.42%,and the specificity was 87.5%(AUC = 0.936,P <0.0001).The critical level of GDF-15 was 292.13 pg/ml,the sensitivity was 80.3%,and the specificity was 92.5%(AUC = 0.925,P <0.0001).The critical level of Wnt-5a is 0.8 ng/ml,the sensitivity is 92.42%,and the specificity is 62.5%(AUC=0.789,P <0.0001).7.Survival study of idiopathic inflammatory myopathy-associated interstitial lung disease: Kaplan-Meier method was used to estimate the cumulative survival probability between the ars-ild group,mda5-ild group and MSA-ILD group,and the cumulative survival rate of iim-ild patients with positive MDA5 antibody was found to be 43%,with significantly reduced survival rate(P =0.001).Conclusion1.Compared with patients with idiopathic inflammatory myopathy with or without lung involvement,that lung involvement patients were older and more likely to have joint pain,fever,mechanic's hand,hypoxemia,high CRP value,low CK level,low AST level,low ALT level,positive for anti-ssa antibody,positive for anti-JO-1 antibody and positive foranti-MDA5 antibody.Meanwhile,The older,mechanic's hand,positive for anti-JO-1 antibody and positive for anti-MDA5 antibody are risk factors for the occurrence of interstitial lung disease associated with idiopathic inflammatory myopathy.2.The imaging of idiopathic inflammatory myopathy-related interstitial lung disease is mainly grid shadow,consolidation shadow,linear shadow and ground glass shadow.The types of iim-ild were mainly NSIP combined with OP,among which ARS antibody-positive ILD patients were mainly NSIP and NSIP combined with OP,MDA5 antibody-positive ILD patients were mainly unclassified CT pattern with consolidation and ground glass shadow,and MSA antibody-negative ILD patients were mainly NSIP.3.Patients with pulmonary involvement in idiopathic inflammatory myopathy with positive anti-MDA5 antibody have high mortality and poor prognosis.4.The diagnostic thresholds of serum KL-6,GDF-15,and Wnt-5a for idiopathic inflammatory myopathy-related lung diseases were 431.3U /ml,292.13 pg/ml,and 0.8 ng/ml,respectively.KL-6,GDF-15,and Wnt-5a were highly sensitive and specific for idiopathic inflammatory myopathy-related lung diseases. |
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