干燥综合症评分最终版

干燥综合征的疾病评价北京积水潭医院-伍沪生SS以淋巴细胞增殖和进行性外分泌腺体损伤为特性的系统性疾病临床特点：温和但可能影响功能的症状：干燥症状、疼痛、疲乏，见于大多患者潜在系统性表现：见于20-40%的患者核心指标：症状（sicca特点、疲乏、健康相关的生活质量）系统损害的活动性（复合的活动指标和实验室检测）现有pSS疾病评价标准分类：1疾病活动度评分（SSDAI、SCAI、ESSDAI）2患者主观评分(PROFAD、SICCA、ESSPRI)3疾病损伤评分（SSDDI、SSDI）ESSPRI的产生EULARSjogren'sSyndromePatientReportedIndex2008.12.4——2009.11.30入组230名SS患者，来自欧洲、南北美洲12个国家21名SS专家参与EULAR的国际合作项目2011年2月研究结果online版首发发表于ARDEULARSjogren'sSyndromePatientReportedIndex(ESSPRI):developmentofaconsensuspatientindexforprimarySjogren'ssyndrome.AnnRheumDis.2011Jun;70(6):968-72.Epub2011Feb22.ESSPRI的实验设计内容4个主要症状：干燥症状、疼痛、躯体疲劳、精神疲倦。每项分值范围0——10分患者认为最需要改善的一项症状疲劳与不适问卷(PROFAD)Sicca症状调查表(SSI)金标准：患者整体评价（PGA）0——10分EULARSjogren'sSyndromePatientReportedIndex(ESSPRI):developmentofaconsensuspatientindexforprimarySjogren'ssyndrome.AnnRheumDis.2011Jun;70(6):968-72.Epub2011Feb22.ESSPRI的实验结果1.多变量回归分析证明，干燥症状、肢体痛、疲劳与PGA符合度好，但是精神疲倦与PGA符合度较差。2.患者认为最需要改善的症状：干燥症状（38.7%）、疲劳（32.2%）、肢体痛（19.6%），最不重要的为精神疲倦3.在干燥症状中，口干、眼干被认为最重要4.ESSPRI最终评分为干燥症状、疲乏、肢体痛三个积分的平均值，范围为0——10分EULARSjogren'ssyndromediseaseactivityindex:developmentofaconsensussystemicdiseaseactivityindexforprimarySjogren'ssyndrome.AnnRheumDis.2010Jun;69(6):1103-9.Epub2009Jun28.Erratumin:AnnRheumDis.2011May;70(5):880.ESSPRI的特性•1.完全由患者主观填表所得，与ESSDAI良好互补•2.与PGA、PROFAD、SSI符合度好（r=0.70、0.73、0.66)，用以评价患者症状的准确性较高。•3.大样本、多中心•4.简单易行，适用于临床EULARSjogren'sSyndromePatientReportedIndex(ESSPRI):developmentofaconsensuspatientindexforprimarySjogren'ssyndrome.AnnRheumDis.2011Jun;70(6):968-72.Epub2011Feb22.PROFAD评分MeasurementoffatigueanddiscomfortinprimarySjogren'ssyndromeusinganewquestionnairetool.Rheumatology(Oxford).2004Jun;43(6):758-64.Epub2004Mar23.SSI评分(siccasymptomsinventory)ValidationoftheSiccaSymptomsInventoryforclinicalstudiesofSjögren'ssyndrome.BowmanSJ,BoothDA,PlattsRG,FieldA,RostronJ;UKSjögren'sInterestGroup.JRheumatol.2003Jun;30(6):1259-66.EULAR的疾病活动度评分标准ESSDAIConstitutionaldomain[3]Pleasebecarefulofnotratingconstitutionalsymptomsnotrelatedtothedisease(suchasfeverofinfectiousorigin,voluntaryweightloss)NoactivityAbsenceofthefollowingsymptomsLowactivityMildorintermittentfever(37.5°-38.5°C)/nightsweatsInvoluntaryweightlossof5to10%ofbodyweightModerateactivitySevereFever(38.5°C)/nightsweatsInvoluntaryweightlossof10%ofbodyweightLymphadenopathydomain[4]NoactivityAbsenceofthefollowingfeaturesLowactivity-Lymphadenopathy≥1cminanynodalregionor≥2cmininguinalregionModerateactivity-Lymphadenopathy≥2cminanynodalregionor≥3cmininguinalregion,-and/orsplenomegaly(clinicallypalpableorassessedbyimaging)Highactivity-CurrentmalignantB-cellproliferativedisorderGlandulardomain[2]Pleasebecarefulofnotratingglandularswellingnotrelatedtothedisease(suchasstoneorinfection)NoactivityAbsenceofglandularswellingLowactivitySmallglandularswellingwith:-enlargedparotid(≤3cm),-orlimitedsubmandibularorlachrymalswelling1ModerateactivityMajorglandularswellingwith:-enlargedparotid(3cm)-orimportantsubmandibularorlachrymalswelling11DistinctionbetweenlimitedandimportantsubmandibularorlachrymalswellingislefttothephysicianjudgmentEULARSjogren'ssyndromediseaseactivityindexArticulardomain[2]Pleasebecarefulofnotratingarticularinvolvementnotrelatedtothedisease,suchasosteoarthritisNoactivityAbsenceofcurrentlyactivearticularinvolvementLowactivityArthralgiasinhands,wrists,anklesandfeetaccompaniedbymorningstiffness(30min)Moderateactivity1to5synovitisamonga28countHighactivity≥6synovitisamonga28countCutaneousdomain[3]Pleasebecarefulofratingas“Noactivity”stablelonglastingfeaturesthatarerelatedtodamageratherthandiseaseactivity,orcutaneousinvolvementnotrelatedtothediseaseNoactivityAbsenceofcurrentlyactivecutaneousinvolvementLowactivityErythemamultiformeModerateactivityLimitedcutaneousvasculitis,includingurticarialvasculitis2,orpurpuralimitedtofeetandankle,orsubacutecutaneouslupusHighactivityDiffusecutaneousvasculitis,includingurticarialvasculitis2,ordiffusepurpuraorulcersrelatedtovasculitis2Limitedcutaneousvasculitisinvolve18%bodysurfacearea;DiffuseCutaneousvasculitisinvolve18%bodysurfaceareaBodysurfacearea(BSA)isdefinedusingtherulesofnines(usedtoassessextentofburns)asfollows:Palm(excludingfingers)=1%BSA;eachlowerlimb=18%BSA;eachupperlimb=9%BSA;torso(front)=18%BSA;torso(back)=18%BSAPulmonarydomain[5]Pleasebecarefulofratingas“Noactivity”stablelonglastingfeaturesthatarerelatedtodamageratherthandiseaseactivity,orrespiratoryinvolvementnotrelatedtothedisease,(tobacco…)NoactivityAbsenceofcurrentlyactivepulmonaryinvolvementLowactivityPersistentcoughorbronchialinvolvementwithnoradiographicabnormalitiesonX-rayOrradiologicalorHRCTevidenceofinterstitiallungdisease3with:Nobreathlessness,Andnormallungfunctiontest.Renaldomain[5]Pleasebecarefulofratingas“Noactivity”stablelonglastingfeaturesthatarerelatedtodamageratherthandiseaseactivity,andrenalinvolvementnotrelatedtothediseaseIfbiopsyhasbeenperformed,pleaserateactivitybasedonhistologicalfeaturesfirstNoactivityAbsenceofcurrentlyactiverenalinvolvement:-Proteinuria0.5g/d,nohematuria,noleucocyturia,noacidosis.-OrlonglastingstableproteinuriaduetodamageLowac