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,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,*,伴肺部损害的自身免疫病,伴肺部损害的自身免疫病,Evidence-Based Medicine,Dept of Rheumatology,Evidence-Based Medicine Dept o,Dept of Rheumatology,CTD-ILD,ILD in established CTD,CTD presenting as ILD,ILD,with features,of autoimmunity,CTD-ILD,有几种类型？,1,Dept of RheumatologyCTD-ILDILD,Dept of Rheumatology,CTD-ILD,ILD,with features,of autoimmunity,UCTD-ILD,Lung-dominant CTD,ILD with autoantibodies,A,formes-frustes,of autoimmune rheumatic,disease,Dept of RheumatologyCTD-ILDILD,Dept of Rheumatology,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,Dept of RheumatologyCTD-ILDInt,Dept of Rheumatology,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),Dept of RheumatologyCTD-ILDInt,Dept of Rheumatology,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,Dept of RheumatologyCTD-ILDInt,Dept of Rheumatology,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,Dept of RheumatologyCTD-ILDInt,RA-ILD,SSc-ILD,IIM-ILD,Other CTD-ILD,CTD-ILD,Dept of Rheumatology,RA-ILDCTD-ILDDept of Rheumatol,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,Dept of Rheumatology,RA-ILDPrevalence:Dept of Rheum,RF,Anti-CCP,Smoking,Genetic factors(,HLA-B40,B54 polymorphisms,),RA-ILD,Biomarkers/risk factors,Dept of Rheumatology,RFRA-ILDBiomarkers/risk fact,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,Dept of Rheumatology,RA-ILDNo RCTs exist to guide p,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,Dept of Rheumatology,RA-ILDThe management of asympt,RA-ILD,3,例,RA-ILD