Clinical Analysis Of Connective Tissue Disease Related Interstitial Lung Disease

ObjectiveAnalyze the clinical types,general conditions,auxiliary examination,treatment and outcomes of CTD-ILD patients,so as to clarify the current situation of clinical diagnosis and treatment in rheumatology,and to improve clinical awareness.MethodsRetrospectively analyze the medical records of patients with diffuse connective tissue disease during 2016.01-2019.06 in the Rheumatology and Immunology Department of the first affiliated Hospital of Suzhou University,which remove other causes of ILD,including general statistics,the types of the primary disease,laboratory examination,autoimmune antibodies,HRCT manifestations,pulmonary function test,lung ultrasound,medication and prognosis.Results143 patients were enrolled,the average age of inpatients was 57.9±11.3 years old,male:female=1:6.9.The main types of secondary ILD were as follows:RA 23 cases,SS 20 cases,SLE 22 cases,SSc 17 cases,IIM 30 cases,IPAF 19 cases,AAV 5 cases and OS 7 cases.There was no significant difference in cough,expectoration,chest stuffiness,Velcro rale between CTD in each group(P>0.05).ESR was increased in 63 patients and CRP in 65 patients.There was no significant difference among CTD groups(P>0.05).The increase of ESR and the decrease of C3,and the simultaneous increase of ESR and IgG had statistical significance(P<0.05).Anti-ENA antibodies were related to each CTD,and there was significant differences in antibodies among different disease groups(P<0.05).There was no significant difference in antibody statistics between patients with IPAF and other specific types of CTD(P>0.05).4 patients of CADM were found to have positive MDA5.HRCT was performed in 143 patients,suggesting interstitial changes(with or without pulmonary infection).132 cases with specific images were counted,mainly for grinding glass shadow,grid shadow,strip shadow,nodular shadows,pleural thickening,honeycomb shadow and pleural effusion.Imaging findings showed no significant difference in CTD among all types(P>0.05).63 patients underwent lung function examination(8 patients failed to perform diffusion function measurement due to incompatibility).Restrictive ventilation dysfunction occurred in 41 cases,obstructive ventilation dysfunction occurred in 3 cases,and diffusion dysfunction occurred in 38 cases,DLCO 65.4±19.0%decreased the most significantly.Lung ultrasound was performed in 16 patients,including SS 7 cases,IIM 4 cases,RA 2 cases,SSc 1 case,IPAF 1 case and OS 1 case.Multiple B-lines(the comet tail sign)and pleural thickening were found in 15 cases by Brightness-mode ultrasound,and "Seashore sign" disappeared on Motion-mode ultrasound.Among the 93 patients who had received cardiac ultrasound and estimated pulmonary artery pressure based on tricuspid regurgitation,15 patients had pulmonary hypertension,including 11 mild,3 moderate and 1 severe.The average pulmonary artery pressure was 44.1±10.2mmHg.A total of 139 patients were treated with glucocorticoid after admission,and 95.0%were given medium to large doses of glucocorticoid(equivalent to prednisone>7.5mg/d).There were 112 patients who were treated with immunosuppresant,including CTX 24,AZA4,MMF 35,HCQ 61,MTX 3,LEF 6,CsA4 and FK 506 4.When the condition is serious,it can be used in combination.41 patients were treated with pirfenidone and 16 with acetylcysteine.The prognosis was improved or not deteriorated in 133 cases,progression or automatic discharge in 10 cases,6 cases mainly caused by respiratory failure.62 cases were complicated with infection,of which 56 were pulmonary infection.The median hospital stay was 13.0±6.1 days.10 patients were hospitalized in 2016,23 in 2017,62 in 2018 and 48 in the first half of 2019.ConclusionThe prevalence of CTD-ILD in our department was mainly in middle-aged women,and most of them had interstitial lung disease caused by IIM,RA and SLE.According to the new definition,the original diagnosis of connective tissue disease or undifferentiated connective tissue disease related interstitial lung disease patients were redefined as"Interstitial pneumonia with autoimmune features",follow-up observation is required to further confirm whether a connective tissue disease.Cough,expectoration and chest shortness of breath were important clinical manifestations.Potential CTD should be considered when lung manifestations occur.The image examination saw more ground glass shadow;Restrictive ventilation dysfunction and diffusion dysfunction often occur in lung function.Pulmonary ultrasound can be used as a radiation free method for the more convenient and non-invasive follow-up of CTD-ILD patients.Glucocorticoid combined with immunosuppressive agents was a common therapy.Pirfenidone was often used in the treatment of fibrosis.After systematic standard treatment,the condition of most patients was under control.