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伴肺部损害的自身免疫病.ppt

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    • 单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,伴肺部损害的自身免疫病,CTD-ILD,ILD in established CTD,CTD presenting as ILD,ILD,with features,of autoimmunity,CTD-ILD,有几种类型?,1,CTD-ILD,ILD,with features,of autoimmunity,UCTD-ILD,Lung-dominant CTD,ILD with autoantibodies,A,formes-frustes,of autoimmune rheumatic,disease,CTD-ILD,Interstitial pneumonia with autoimmune features,(IPAF),Presence of an interstitial pneumonia(by HRCT or surgical lung biopsy),and,Exclusion of alternative aetiologies,and,Does not meet criteria of a defined CTD,and,At least one feature from at least two of these domains:,A.Clinical domain B.Serologic domain C.Morphologic domain,CTD-ILD,Interstitial pneumonia with autoimmune features,(IPAF),A.Clinical domain,Distal digital fissuring(,i.e.,“,mechanic hands,”,),Distal digital tip ulceration,Inflammatory arthritis,or,polyarticular morning joint stiffness,60 min,Palmar telangiectasia,Raynaud,s phenomenon,Unexplained digital oedema,Unexplained fixed rash on the digital extensor surfaces(Gottron,s sign),CTD-ILD,Interstitial pneumonia with autoimmune features,(IPAF),B.Serologic domain,ANA1:320,Rheumatoid factor,2 upper limit of normal,Anti-CCP,Anti-dsDNA,Anti-Ro(SS-A),Anti-La(SS-B),Anti-ribonucleoprotein,Anti-Smith,Anti-topoisomerase(Scl-70),Anti-tRNA synthetase,Anti-PM-Scl,Anti-MDA-5,CTD-ILD,Interstitial pneumonia with autoimmune features,(IPAF),C.Morphologic domain,Suggestive radiology patterns by HRCT:,a.NSIP;b.OP;c.NSIP with OP overlap;d.LIP,Histopathology patterns or features by surgical lung biopsy:,a.NSIP;b.OP;c.NSIP with OP overlap;d.LIP e.Interstitial lymphoid aggregates with germinal centres f.Diffuse lymphoplasmacytic infiltration,Multi-compartment involvement(in addition to interstitial pneumonia):,a.Unexplained pleural effusion or thickening b.Unexplained pericardial effusion or thickening c.Unexplained intrinsic airways disease,d.Unexplained pulmonary vasculopathy,RA-ILD,SSc-ILD,IIM-ILD,Other CTD-ILD,CTD-ILD,RA-ILD,Prevalence:,3.5%-41%in RA(,方法,时间,),Histopathological types,:,UIP and NSIP are the most common types,Other subtypes include OP,AIP or DAD,LIP,and DIP,RF,Anti-CCP,Smoking,Genetic factors(,HLA-B40,B54 polymorphisms,),RA-ILD,Biomarkers/risk factors,RA-ILD,No RCTs exist to guide pharmacological therapy specifically in RA-ILD.,Steroids were often used as first line agents with highly variable results.,NSIP and OP-ILD subtypes are steroid responsive.NSIP subtype can be managed with aggressive steroid therapy,Other regimen that includes pulsed CTXiv and steroids.Pts who respond well are maintained on oral AZA and steroids,treatment,RA-ILD,The management of asymptomatic ILD in RA pts remains unclear.It is not known whether treatment will alter the course of the disease in these cases,MTX has been established as a source of pneumonitis in 0.5%to 14%of treated pts.But MTX has not been conclusively shown to worsen RA-ILD directly,LEF,another DMARD,has similar associations with ILD reported in the literature.,treatment,RA-ILD,3,例,RA-ILD,用,MMF,治疗一年,,ILD,及关节炎均有改善,Saketkoo LA,et al.RA-ILD,:MMF as an antifibrotic and DMARD.,Arch Intern Med,2008:168:1718.,Chang HK,Park W,Ryu DS.Successful treatment of progressive rheumatoid ILD with cyclosporine:a case report.,J.Korean Med.Sci.,17,270273(2002).,Cohen JM,et al.Interstitial pneumonitis complicating RA.Sustained remission with azathioprine therapy.,Chest,72,521524(1977).,MMF,CosA,AZA,treatment,SSc-ILD,CTD-ILD,SSc-ILD,Prevalence:occurring to some degree in about 80%of patients with diffuse cutaneous scleroderma and in 20%of patients with limited skin disease.,Histopathological types:The most common pattern is nonspecific interstitial pneumonia(NSIP),the others are rare.,Diffuse skin involvement,Esophageal involvement,Anti-SCL-70,vs,anti-RNP,SSc-ILD,Biomarkers/risk factors,SSc-ILD,N Engl J Med 2006;354:2655-66(USA).,Treatment:,CTX,At 13 clinical centers,enrolled 158 patients with SSc.Patients received oral CTX(2 mg/kg/D)or matching placebo for one year and were followed for an additional year.The primary end point was the forced vital capacity(FVC)at 12 months,after adjustment for the baseline FVC.,SSc-ILD,SSc-ILD,SSc-ILD,CONCLUSIONS,One year of oral CTX in symptomatic SSc-ILD had,a significant but modest beneficial effect on,:,lung function,Dyspnea,Thickening of the skin,Health-related quality of life.,The effects on lung function were maintained through the 24 months of the study,SSc-ILD,Hoyles RK,et al.,A multicenter,prospective,randomized,double-blind,placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma.,Arthritis Rheum.,54,3962(2006)UK.,45 patients with SSc-ILD who were randomized to receive prednisolone and six CTX infusions or placebo.The estimated relative treatment effect in this study revealed a mean improvement of,4.19%,of FVC,but DLCO was not improved.,Treatment:,CTX,SSc-ILD,Zamora evaluated 17 。

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