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1、CaseReview1现病史患者,男,48岁,杭州人,经商患者半月余前无明显诱因下出现上腹痛,不剧可忍,持续2小时后缓解,无肩背部放射痛,无发热畏寒,无恶心呕吐,无肛门停止排气排便,无胸闷心悸,无呕血黑便,此后上腹部隐痛每天发作2-3次,与进食、排便无明显相关,半月来患者自觉症状加重,上腹部持续隐痛不适,阵发性加重,有时较难忍,发作频率增加,遂来我院就诊。自病以来,神清,精神可,胃纳可,睡眠一般,大小便无殊,体重无明显减轻。2既往史/个人史/家族史既往体健否认食物药物过敏史否认长期服药史吸烟史:1包/天20年,否认饮酒史家人体健3体格检查神清,精神偏软,生命体征平稳,浅表淋巴结未及肿大,两肺呼
2、吸音清,未闻及啰音,心律齐,未闻及杂音,腹软,上腹部及脐上轻压痛,无反跳痛,肠鸣音6次/分,双下肢无水肿。4辅助检查腹部CT:肝内小钙化灶,右肾小结石,右肾盂旁囊性灶,肾盂旁囊肿可能。胃镜:胃腔内大量食物残留,影响观察;病理:慢性炎,灶性活动性,灶性肠化,幽门螺杆菌:阴性5入院诊断腹痛:消化性溃疡?消化道肿瘤?胆管结石?6诊治经过7诊治经过予屈他维林、泮托拉唑静滴,患者症状无缓解BUS:肝内钙化灶,右肾小结石8腹痛腹痛+嗜酸粒细胞增多嗜酸粒细胞增多=?9鉴别诊断Yan and Shaffer. Gut 2009.10诊治经过复查胃镜11诊治经过术前免疫、甲状腺功能、尿常规、PPD试验、大便培养
3、、CXR无殊开瑞坦1#poqd,2天后症状缓解拒绝骨髓穿刺检查,带药出院12诊治经过追问病史:起病前食用花生出院后2周门诊随访,症状未再发,嗜酸性粒细胞计数恢复正常13病理诊断14病理诊断胃镜病理:送检“十二指肠降部”及“球部”组织,镜示小肠粘膜内见大量淋巴浆细胞聚集,并见较多嗜酸性粒细胞浸润,有处高倍达50个以上,可见脱颗粒现象,未见血管炎,未见肉芽肿,未见隐窝脓肿,间质水肿不明显。送检“胃窦”、“胃角”及“胃体”组织,镜示胃粘膜呈慢性炎改变伴淋巴细胞灶性聚集,可见部分嗜酸性粒细胞散在分布。HP阴性15最后诊断原发性嗜酸性粒细胞性胃肠炎原发性嗜酸性粒细胞性胃肠炎Talley诊断标准:(1)有
4、胃肠道症状(2)病理证实胃肠道一处或多处嗜酸性粒细胞浸润(3)排除寄生虫感染和胃肠道外嗜酸性粒细胞增多疾病16原发性嗜酸性粒细胞性胃肠炎流行病学过敏性胃肠病(Allergicgastroenteropathy)最早于1937年由Kaijser提出发病率约为1/10万好发年龄30-60岁发病男女比例1.4:125%-75%的患者有过敏病史17Yan and Shaffer. Gut 2009.发病机制18分型与临床表现19辅助检查75%-80%的患者外周血嗜酸粒细胞升高IgE水平可以升高或者正常胃镜下无特异性表现:粘膜充血、红斑、水肿、结节、蠕动异常影像学表现各异20病理表现灶性分布,最常累及胃
5、和十二指肠嗜酸性粒细胞浸润(20-50/HPF)嗜酸性粒细胞可侵及粘膜上皮层、固有肌层、浆膜层微脓肿形成脱颗粒现象21诊断标准Talley诊断标准:诊断标准:(1)有胃肠道症状;(2)病理证实胃肠道一处或多处嗜酸性粒细胞浸润;(3)排除寄生虫感染和胃肠道外嗜酸性粒细胞增多疾病Leinbach诊断标准:诊断标准:(1)进食特殊食物后出现胃肠道症状;(2)外周血嗜酸粒细胞增多;(3)病理证实胃肠道嗜酸性粒细胞浸润除外继发性的胃肠道嗜酸粒细胞浸润除外继发性的胃肠道嗜酸粒细胞浸润22治疗避免接触过敏原:花生、大豆、鸡蛋、牛奶、花粉激素:泼尼松20-40mg/天,6-8周后减量抗过敏药物:色甘酸钠、酮替
6、芬、孟鲁司特免疫抑制剂:硫唑嘌呤手术:胃肠道穿孔、梗阻23小结24THANKS25Althoughconsideredidiopathic,anallergicmechanismmaybeinvolvedasmostpatientsexhibitincreasedtotalIgEandfood-specificIgElevels.SecondarydisorderslinkedtoEGEincludethehypereosinophilicsyndrome(HES),coeliacdisease,Crohnsdisease,vasculitis(ChurgStrausssyndrome,pol
7、yarteritisnodosa),connectivetissuedisease(scleroderma),infection(Helicobacterpylori,parasites)anddruginjury/hypersensitivity.26ThediagnosisofEGEmaybeelusive.Symptomsarenon-specific.Peripheraleosinophiliaisvariable;theeosinophilcountisnormalin25%ofpatients.Atopyandallergiesareassociatedin2575%ofcases
8、.27MucosaldiseaseisthemostreadilydiagnosedformofEGE,giventhatendoscopycandirectlyvisualiseanymucosalchangesandacquirebiopsies.Endoscopicfeaturesareneverthelessrathernon-pecific:thickenedfolds,erythema,friability,nodularity,andabnormalperistalsis.28修正诊断腹痛:寄生虫感染?嗜酸粒细胞性胃肠炎?高嗜酸粒细胞综合征?29预后容易复发30自然病程免疫抑制剂
9、的疗程激素的减量法3132Asperthedefinition,patientswithEGIDsandsustainedbloodeosinophiliaexceeding1500cells/mmwouldhaveHES.Therefore,routinesurveillanceofthecardiorespiratorysystemiswarranted,especiallyifthepatientshaveextragastrointestinalmanifestations.33Theysuggestedthefollowingdiagnosticcriteria:(1)thepres
10、enceofgastrointestinalsymptoms,(2)biopsiesshowingeosinophilicinfiltrationofoneormoreareasofthegastrointestinaltractfromesophagustocolon,orcharacteristicradiologicfindingswithperipheraleosinophilia,(3)noevidenceofparasiticorextraintestinaldisease.34Thegastrointestinaltractisthemainnonhematopoieticorg
11、anwhereeosinophilsresideinthehealthystate.Eosinophilsarenormallypresentinthelaminapropria,butthenumberofeosinophilsregardedaspathologicalforvarioussitesalongthegastrointestinaltractisdebated;thehighestconcentrationsarefoundinthececumandappendix.Withinthegastrointestinaltract,35Inpublishedreports,the
12、mostfrequentlyaffectedorganswerethesmallintestineandstomach.ManypatientswithEGhaveahistoryofseasonalallergies,foodsensitivities,eczema,asthma,andatopy.Talleyetal.reportedahistoryofallergyin20of40patientswithEG.ThemostcommonfoodsreportedtobepositivebySPTincludecommonfoodallergenspeanuts,eggs,soy,cowm
13、ilk,andwheatinadditiontobeans,rye,andbeef.36HESisanidiopathicconditiondefinedasmarkedperipheraleosinoiliaexceeding1500cells/lthatpersistsforatleas6months;thereisorgandysfunction.Target-organdamagemediatedbyeosinophilsishighlyvariablemongpatients,withinvolvementofskin,heart,lung,centralandperipheraln
14、ervoussystemsinmorethan50%ofcases.37therapeuticstrategy.First,theyrecommendedspecifiallergyavoidance(airborneanddietary).IfthisfailedtoimproveEGIDpathology,thentheyrecommendedglucocorticoidtherapy,firststartingwithtopicaldeliveryandthenconsideringsystemicdelivery.Theyalsoadvocatedelementaldiettrials
15、aimedatavoidingallproteinantigenexposure.38Mostpatientsreceiveprednisoloneindosesfrom20to40mg/dayfor68weekswithvariousschemesofdosetapering.Otherdrugs,suchassodiumchromoglycateandketotifen(antihistamineandmastcell-stabilizingagents),suplatasttosilate(antiallergicdrugthatsuppressescytokinesproduction
16、),andmontelukast(leukotrienereceptorantagonist),werefoundtobeeffectiveinthemanagementaswellassteroid-sparingagentsinsomecasereportsbutnotinothers.Surgicaltreatmentisrequiredforpatientswithintestinalperforationand/orobstruction.39theideathattheeosinophilsinthedigestivetractiscausedbytheexposureofpatientstodietaryorairborneantigenstheconditioncanresolveitselfpermanentlyovertime,butitismorelikelytobecharacterizedbyrecurrentrelapses.40