嗜酸性粒细胞肠炎

1CaseReport

--EosinophilicGastroenteritis

嗜酸性粒细胞肠炎编辑ppt2A52-year-oldmanpresentedtotheemergencydepartmentwithabdominaldiscomfort.Hissymptomsbegan5weeksearlier,whennonradiatingdiscomfortdevelopedintheepigastricregion.Thepainworsenedaftereatingandwasassociatedwithnauseaandbloatingthatwerepartiallyrelievedbyeructation.Twoweeksbeforepresentation,thepatient’spainbecamemorelocalizedtotheperiumbilicalareaandwasaccompaniedbyearlysatietyandmultipleepisodesofvomiting.Hehadnofever,diarrhea,tenesmus,melena,orhematochezia.

一名52岁男性因腹部不适急诊就诊。5周前开始上腹痛，疼痛不呈放射性，疼痛于进食后加重并伴恶心与腹胀，在打嗝后部分缓解。来院两周前腹痛集中于脐周伴早饱伴呕吐。不伴发热，腹泻，里急后重、黑便、便血。编辑ppt3Themedicalhistoryincludedmildintermittentasthmathatwasdiagnosed20yearsearlierandrequiredonlyveryoccasionaluseofinhaledbeta-agonists,obstructivesleepapnea,hypertension,dyslipidemia,type2diabetesmellitusthatwaswellcon-trolledwithoralagents,hypothyroidism,andgout.Adiagnosisofchronicidiopathicangioedema–urticariawasestablished11yearsearlier,afterthepatientpresentedwithsporadicepisodesofcutaneousswellingandhives.Skintestingdidnotidentifyanallergictrigger,andlevelsofserumcomplementwerenormal.Symptomswerealleviatedwithcetirizineandranitidine..病史为诊断为轻微间断哮喘20年，偶尔使用吸入性贝塔受体阻滞剂。阻塞性睡眠呼吸障碍综合征，高血压，高血脂，2型糖尿病控制良好现口服降糖药，甲状腺功能减退，痛风。11年确诊为特发性血管性荨麻疹，伴间断出现的皮肤水肿及荨麻疹，皮肤测试未发现过敏原，血清学检查补体正常，症状在使用西替利嗪与雷尼替丁后缓解。编辑ppt4Othermedicationsincludedlisinopril(initiated18monthsbeforepresentation),simvastatin,metformin,levothyroxine,andcolchicine.Thepatientwasmarried,withtwochildren,andworkedasapension-fundmanager.Hedrankfivebeersandsmokedonepackofcigarettesweekly.Hedidnotuseillicitdrugs.HehadtraveledtoPanama5yearsbeforepresentationandtoScotland1yearbeforepresentation.其它用药史包括口服莱诺普利18月。辛伐他汀，二甲双胍，左旋甲状腺素，秋水仙碱。患者已婚，育有2个孩子，工作为养老金基金经理，5瓶啤酒/周，1包烟/周。无药物滥用史。5年前去巴拿马旅游，1年前去过苏格兰。编辑ppt5Physicalexaminationrevealedawell-nourishedmanwhowasnotinacutedistress.Histemperaturewas36.1°C,heartrate77beatsperminute,bloodpressure106/60mmHg,respiratoryrate20breathsperminute,andoxygensaturation98%whilehewasbreathingambientair.Thescleraewereanicteric.Theneckwassupple,withoutpalpablelymphadenopathyorthyromegaly.Thelungswereclearonauscultation,andthecardiacexaminationwasnormal.Theabdomenwasdistended,butwithoutbulgingflanksorfluidwave.Bowelsoundswerenormal.体检示患者发育良好，无痛苦表情。体温36.1度，心率77次/分，血压106/60，呼吸20次/分，血氧饱和度98%（呼吸空气）。巩膜无黄染，颈软，未触及肿大淋巴结及甲状腺。听诊呼吸音清，心脏体检正常。腹胀，无侧腹膨隆及液波震颤。肠鸣音正常。编辑ppt6Palpationoftheabdomenrevealedmild,diffusetendernesswithoutrebound,guarding,ororganomegaly.Noluminalirregularitiesweredetectedonrectalexamination,andaguaiactestofstoolwasnegative.Thepatient’sarmsandlegswerewarmandwellperfused.Skinandneurologicexaminationswereunremarkable.触诊腹软，无腹肌紧张、反跳痛，未触及器官肿大。直肠检查未发现肠腔狭窄。粪隐血试验隐性。四肢温暖，灌注正常，皮肤神经检查正常。编辑ppt7Themostnotablelaboratoryfindingisthehighproportionofeosinophilsat39%,withacalculatedabsoluteeosinophilcountofabout3700cellspercubicmillimeter.最显著的实验室检查异常是嗜酸性粒细胞比例增高，嗜酸性粒细胞绝对值3700/立方厘米。编辑ppt8Thepatientwasadmittedforfurtherevaluation.Measurementofstrongyloidesantibodiesbyenzyme-linkedimmunosorbentassay(ELISA)revealedacountoflessthan1ELISAunit.Theresultsofasinglestoolstudyforovaandparasiteswasnegative,andserumantineutrophilcytoplasmicantibodieswereundetectable.患者进行了进一步检查，ELISA法查粪类圆线虫钩虫抗体阴性。大便查虫卵和寄生虫阴性，血清抗中性粒细胞胞质抗体未检出。编辑ppt9Axialimages(PanelsAandB)andacoronalimage(PanelC)showcircumferentialwallthickeninginalongsegmentofjejunum(PanelA,arrows).Muralstratificationwithrelativelyincreasedenhancementofthemucosaandserosasuggestsbowel-walledema(PanelsBandC,arrows).Amoderatevolumeofascitesisseeninthesmall-bowelmesentery,perihepaticspace,andpelvis(PanelC,asterisks).一段空肠肠壁环形增厚，粘膜层与浆膜层可见强化，说明有肠壁水肿。肠系膜周围、肝周、脾周及盆腔可见中等量腹水。编辑ppt10编辑ppt11编辑ppt12编辑ppt13Ultrasound-guidedparacentesisfailedtoprocureasciticfluidforanalysis.Esophagogastroduoden­oscopywithpushenteroscopyrevealedanormal-appearingesophagus,stomach,pylorus,duodenum,andjejunum.Analysesofmultiplerandombiopsyspecimensobtainedfromthejejunumrevealednormalsmall-intestinalmucosa.超声引导穿刺腹水检查未成功。胃镜及肠镜检查示食管、胃、幽门、十二指肠、空肠正常。多处空肠活检示正常小肠黏膜。编辑ppt14Anexploratorylaparotomywasperformed,andafull-thicknessbiopsyspecimenofthejejunumwasobtained.Thespecimenshowedinfiltrationofthemuscularispropriaandsubserosabysheetsofeosinophils.Asciticfluidobtainedatthetimeofsurgerycontained13,200whitecellspercubicmillimeter,with68%eosinophils,andnocellsthatappearedtobemalignant.患者行剖腹探查术并取空肠活检示黏膜下层及固有肌层嗜酸性粒细胞浸润。术中获得腹水检查白细胞13200/立方厘米，嗜酸性粒细胞百分比68%，未发现恶性细胞。编辑ppt15编辑ppt16编辑ppt17Afull-thicknessbiopsyspecimenofthejejunum(PanelA,hematoxylinandeosin)showsinfiltrationofthemuscularispropriaandsubserosabysheetsofeosinophils,withamarkedlythickenedandedematoussubserosa.Ahigh-powerviewofthesubserosa(PanelB)showsnumerouseosinophils,whicharecharacteristicallydeeppinkonstainingwithhematoxylinandeosin.空肠全层活检示固有肌层及黏膜下层嗜酸性淋巴细胞浸润，黏膜下层严重水肿，可见多量嗜酸性粒细胞，苏木精伊红染色呈深红色。编辑ppt18Eosinophilicgastroenteritiswasdiagnosed,andtreat-mentwith60mgofprednisoneorallyperdaywasinitiated,withrapidanddramaticreliefofsymptomsandreductionofperipheraleosinophilia.Thepatientwasslowlyweanedfromtheprednisoneoverthecourseof4months,withoutdiseaserecurrence.Sixmonthsafterhisdiagnosis,heremainssymptom-free,withanormaleosinophilcount,andcontin-uestobefollowedclosely.患者被诊断为嗜酸性粒细胞肠炎。给予患者60mg每天强的松口服，患者症状迅速缓解外周血嗜酸性粒细胞减少。4个月强的松治疗后无复发，6个月后复查嗜酸性粒细胞计数正常，并继续随访。编辑ppt19Eosinophilicgastroenteritisischaracterizedbyaneosinophil-richinflammationinthewallofthestomachorsmallbowel.Beforethisdiagnosisisestablished,itisnecessarytoruleoutothercausesofeosinophilia,suchasdrugreaction,parasiticinfection,andcancer.嗜酸性粒细胞肠炎是胃壁或小肠壁嗜酸性粒细胞炎性浸润的疾病。需要与其他导致嗜酸性粒细胞增多的病因鉴别，例如药物反应、寄生虫感染及癌症。编辑ppt20Inaddition,theinvolvementofotherorgansmustberuledout,sincemultiorganinvolvementindicatesthepresenceofanalternativehypereosinophilicsyndrome,suchaseosinophilicleukemia,hypereosinophilicsyndrome,ortheChurg–Strausssyndrome.2其他器官的累及也应排除，多器官累及可能是其他的高嗜酸性粒细胞综合征，比如嗜酸性粒细胞白血病、高嗜酸性粒细胞综合征，变性肉芽肿性血管炎。编辑ppt21Ifthediagnosisofeosinophilicgastroenteritisissuspected,furtherinvestigationtoruleoutotherhypereosinophilicsyndromesshouldroutinelyincludeaperipheral-bloodsmear,serumlevelsoftryptaseandvitaminB12，andachestradiograph.1Todetectmyocardialdamage,aserumtroponinassayandanechocardiographicstudyshouldbeperformed.如果不能确诊，影星进一步检查如外周血涂片、血清类胰蛋白酶及维生素B12、以排除其他高嗜酸性粒细胞综合征。胸部平片来排除心肌损害，肌钙蛋白水平及超声心动图也应进行。编辑ppt22Forpatientswitheosinophilicgastroenteritis,presentingsymptomstypicallyvaryinaccordancewiththelayerofbowelwallinvolved.嗜酸性粒细胞肠炎的患者症状表现根据肠壁浸润的层不同也不同。编辑ppt23Mucosa-predominantdiseaseismanifestedasnonspecificabdominaldiscomfort,malabsorption,andprotein-losingenteropathy,whereasmuscularis-predominantdiseaseleadstosymptomsofobstruction,suchasearlysatiety,bloating,andnausea.黏膜型表现为不典型的腹部不适，吸收障碍，蛋白质丢失导致的肠下垂，肌层型导致梗阻症状、早饱、腹胀、恶心。编辑ppt24Incontrast,subserosa-predominantdiseasemorecommonlyresultsineosinophilicascitesandmarkedlyelevatedcountsofeosinophilsinperipheralblood.浆膜型主要症状是嗜酸性粒细胞的腹水和外周血嗜酸性粒细胞显著增高。编辑ppt25Thispatienthadinvolvementofboththemuscularisandsubserosallayersofthesmallbowel,withthecharacteristicobstruc-tivesymptoms,eosinophilicascites,andelevatedeosinophilcountinperipheralblood.该患者同时有肌层及浆膜层的累及，有梗阻症状和嗜酸性粒细胞腹水，外周血嗜酸性粒细胞显著增高。编辑ppt26Mucosalbiopsiesarefrequentlyunrevealinginpatientswitheosinophilicgastroenteritisinvolvingthedeeperlayersofthebowelwall;moreinvasiveproceduresareneededtoconfirmthediagnosis.浆膜型患者肌层活检不能检出嗜酸性粒细胞肠炎，需要更侵略性的检查手段。编辑ppt27Thecauseofeosinophilicgastroenteritisisnotwellunderstood.Althoughmanypatientshaveahistoryofallergicdisease,suchasasthma,allergicrhinitis,oratopy,theextentoftheroleplayedbyfoodallergiesinthepathogenesisofeosinophilicgastroenteritisremainscontroversial.嗜酸性粒细胞肠炎的病因未明，很多患者都有过敏性疾病的病史，例如哮喘、过敏性鼻炎或过敏体质。食物作为过敏原引起嗜酸性粒细胞肠炎的发病机理仍有争议。编辑ppt28Skin-pricktestingcanbeperformedwithapanelofallergenstofacilitatedirectedtrialsoffoodavoidance皮肤针刺实验可以检出过敏原，患者可在饮食中规避这些过敏原。编辑ppt29Ifsymptomspersistoraresevere,treatmentwithcorticosteroidsisrecommended.Whencorticosteroidsarerequired,adoseof20to40mgofprednisonedailyisthetypicalstartingdose,whichisthentaperedoverthecourseofseveralmonths.如果症状