惡性高血壓與肺出血

惡性高血壓與肺出血第1頁/共25頁2IgA腎病與肺出血第2頁/共25頁3Pulmonarycapillaritisandalveolarhemorrhage.Updateondiagnosisandmanagement.

Pulmonaryvascularinflammatorydisordersmayinvolveallcomponentsofthepulmonaryvasculature,includingcapillaries.Theprincipalhistopathologicfeaturesofpulmonarycapillaritisincludecapillarywallnecrosiswithinfiltrationbyneutrophils,interstitialerythrocytes,and/orhemosiderin,andinteralveolarseptalcapillaryocclusionbyfibrinthrombi.Immunecomplexdepositionisvariablypresent.Patientsoftenpresentclinicallywithdiffusealveolarhemorrhage,whichischaracterizedbydyspneaandhemoptysis;diffuse,bilateral,alveolarinfiltratesonchestradiograph;andanemia.PulmonarycapillaritishasbeenreportedwithvariablefrequencyandseverityasamanifestationofWegener'sgranulomatosis,microscopicpolyarteritis,systemiclupuserythematosus,Goodpasture'ssyndrome,idiopathicpulmonaryrenalsyndrome,Behcet'ssyndrome,Henoch-Schonleinpurpura,IgAnephropathy,antiphospholipidsyndrome,progressivesystemicsclerosis,anddiphenylhydantoinuse.第3頁/共25頁4Aclinicopathologicstudyof34casesofdiffusepulmonaryhemorrhagewithlungbiopsyconfirmation.

34patientswithbiopsy-confirmeddiffusepulmonaryhemorrhage(DPH).DPHsyndromeswereencountered:antibasementmembraneantibodydisease(fourcases);idiopathicpulmonaryhemorrhage(fourcases);WG(fivecases);probableWG(sixcases);systemicnecrotizingvasculitisotherwiseunclassified(threecases);systemiclupuserythematosus(twocases);rheumatoidarthritis(onecase);seronegativejuvenilerheumatoidarthritis(onecase);IgAnephropathy(onecase);idiopathicglomerulonephritis(twocases--onewithandonewithoutimmunecomplexes);andunclassifiedpulmonary-renalsyndromes(fivecases).Capillaritiswasfoundinlungbiopsysamplesfrom30ofthe34patients(88%)andincludedpatientswitheverytypeofDPHsyndrome.AmJSurgPathol.1990Dec;14(12):1112-25.第4頁/共25頁5Pulmonaryhemorrhage.AfatalmanifestationinIgAnephropathy.

TwopatientswithasymptomaticIgAnephropathy(IgAN)andathirdpatientwithchronicrenalfailureduetoIgANdiedfollowingarecentonsetofdyspnea,hemoptysis,andpulmonaryinfiltrates.Inallcases,thecauseofdeathwasrespiratoryfailureattributedtoeitherbronchopneumoniaorpulmonaryedema.Inallthreepatients,thediagnosesofIgANandidiopathicpulmonaryhemorrhagewereestablishedatpostmortemexamination.Acutealveolarhemorrhagewaspresentintwopatients.Allthreepatientshadheavyalveolarhemosiderin-ladenmacrophages,andcapillaritiswasrecognizedintwoofthem.Theysuggestedanimmunecomplex-mediatedpulmonaryinjurythatwaspossiblyrelatedtothesystemicnatureofIgAN.

ArchPatholLabMed.1994May;118(5):542-6.第5頁/共25頁6IgA腎病與惡性高血壓第6頁/共25頁7惡性高血壓的臨床特征血壓明顯升高,通常舒張壓>130mmHg(16.9kPa);廣泛累及全身小動脈,導(dǎo)致中樞神經(jīng)系統(tǒng)、心、腎等臟器受損,其中以腎臟損害最為顯著;眼底改變包括視網(wǎng)膜出血、棉絮狀滲出及視乳頭水腫等;如不及時治療則預(yù)后不佳,多死于尿毒癥。第7頁/共25頁8惡性高血壓的病因第8頁/共25頁9第9頁/共25頁10

Prognosticfactorsinimmunoglobulin-AnephropathyNinetyeightadultpatientswithdiagnosisofprimaryIgAnephropathy.Outof98patient，64(65.3%)weremen.Meanageofpresentationwas25.7years.Thepredominantrenallesionsincludednephroticsyndromein25(25.5%),rapidlyprogressiverenalfailureandacceleratedmalignanthypertensionin21(21.4%)each,chronicrenalfailurein13(13.3%),hypertensioninnine(9.2%)haematuriainfive(5.1%)andacuterenalfailureinfour(4.1%).Sixty(61%)hadrenalfailureatdiagnosis.Age>25years,glomerularhistologyofHasssubclassVandinterstitialfibrosisweresignificantfactors.Forty(48.2%)(IR)patientsdevelopedESRFduringfollowup.JAssocPhysiciansIndia.2002Nov;50:1354-9.

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MalignantoracceleratedhypertensioninIgAnephropathy.66adultpatientsdiagnosedashavingIgAnephropathybyrenalbiopsy24(36%)werehypertensivewhenfirstseen.Ofthesehypertensivepatients,10(15%)hadmalignantoracceleratedhypertension.Allpatientsbutoneweremaleandhadnoknowledgeoftheirrenaldiseaseandsoughtmedicaladviceforsymptomsduetohypertension.Fivepatientshadnohistoryofgrosshematuria.Histologicalvascularfindingsshowed,inthreeproliferativeendarteritisandfibrinoidnecrosis,infivearteriolosclerosisandintwovascularhypertrophy.Inspiteofgoodbloodpressurecontrol,sixpatientsreachedterminaluremiawithinamaximumof14months.Patientswiththisassociationreachendstagerenalfailureinashortperiodoftime.

ClinNephrol.1987Jan;27(1):1-7.第11頁/共25頁12“Malignant”IgAnephropathy（1）MostpatientswithmesangialIgAnephropathywhorunaprogressivecourseusuallydosooveraperiodof10to20years.Thispaperdescribesthecourseofthreeyoungmenwithsimilarpresentingfeaturesandbiopsyfindingswhoprogressedtoend-stagerenalfailureinlessthan4yearsfrompresentation,eventhoughinitiallyallhadserumcreatininelevelsthatwereinthenormalrange.

AmJKidneyDis.1985Jan;5(1):42-6.第12頁/共25頁13“Malignant”IgAnephropathy（2）Theypresentedwithmacroscopichematuria,whichhaspreviouslybeenregardedasanindicatorofafavorableprognosis,andallthreehadloinpain,constantlyelevatedurinaryerythrocytecounts,andcrescentsinrenalbiopsies.Intwocases,treatmentappearedtobeassociatedwithstabilizationofrenalfunction,butdeteriorationtoend-stagerenalfailureoccurredrapidlyaftertreatmentwasceased.AmJKidneyDis.1985Jan;5(1):42-6.

第13頁/共25頁14IdiopathicIgAnephropathypresentingasmalignanthypertension.

ThreecasesarereportedofidiopathicIgAnephropathy(Berger'sdisease)presentingasmalignanthypertension,withnodatasuggestingtheunderlyingglomerulopathy,whichwasuncoveredonlyafterrenalbiopsywasperformed.Commentsaremadeonthevalidityofthepathologicaldiagnosis,thepossiblepathogeneticsequenceoftheassociation,andontheeventualrisksandbenefitsderivedfromperformingrenalbiopsyinsuchpatients.

AmJNephrol.1986;6(6):482-6.第14頁/共25頁15惡性高血壓與肺出血第15頁/共25頁16張曉亮查房復(fù)習(xí)男，35歲，C800489入院日期：2003-8-1主訴：雙小腿水腫1年，咯血5月，憋氣3月，加重10余天。第16頁/共25頁17現(xiàn)病史

1年前無明顯誘因出現(xiàn)雙小腿可凹性水腫。間斷頭痛，未測血壓。5月前咳少量痰，帶血絲，逐漸加重有小血塊。并出現(xiàn)憋氣，夜尿增多，夜間尿3-4次，尿量無減少，尿中泡沫增多，雙下肢腫較前明顯加重。03-6月初憋氣加重，夜間不能平臥，雙小腿水腫明顯，晨起后自覺眼瞼浮腫，并出現(xiàn)皮膚、鞏膜較前變黃。第17頁/共25頁18第一次住院情況查體：血壓240/130mmHg,鞏膜黃染，雙下肢可凹性水腫（++）尿常規(guī)PRO5g/L，BLD250/UL；尿蛋白4.96g/d，GFR22.7ml/min，免疫指標（-）。6-10血氣：I型呼衰。6-16腎穿“惡性高血壓腎損害”；胸片、胸部CT：雙肺多發(fā)滲出實變性病變，右側(cè)包裹性胸腔積液；肺V/Q顯像：不支持肺栓塞。心臟彩超：左室肥厚，左房增大，射血分數(shù)57%，輕度肺動脈高壓。入院后Scr增高，1.9~4.5mg/dl，6-15開始甲強0.5g/d*3天沖擊，后改為強的松60mg/日。呼吸科會診考慮咯血為高血壓所致。予降壓藥治療后，血壓控制至140/90mmHg，憋氣等癥狀緩解，強的松減至15mg/天，Cr3.3mg/dl后6-30出院。出院后半月內(nèi)逐漸停用強的松。

第18頁/共25頁19第二次住院情況7月18日再次出現(xiàn)頭痛、憋氣、痰中帶血，浮腫加重，夜間不能平臥。急診查T38.2℃，BP185/87mmHg，SCr5.8mg/dl，胸片：雙肺大片高密度影，肺水腫可能；血氣：PO245.8mmHg，SO283.6%，予CPAP輔助呼吸及抗感染治療，持續(xù)硝普鈉、壓寧定等控制血壓。肺活檢示肺左下葉基底段慢性炎，肺泡腔內(nèi)有較多吞噬含鐵黃素的吞噬細胞。臥位PRA9.4mug/ml，ATⅡ409.6uug/ml，Ald21.7mug/dl24小時尿CA：NE16.43ug/d；E3.43ug/d;DA48.77ug/d24小時尿UFC57.8ug/d第19頁/共25頁20診斷：

惡性高血壓急性腎功能衰竭急性左心功能衰竭高血壓視網(wǎng)膜病變Ⅳ期

肺內(nèi)病變性質(zhì)未明

Ⅰ型呼吸衰竭肺水腫肺泡出血合并感染可能性大肺結(jié)核不除外

2型糖尿病結(jié)節(jié)性甲狀腺腫

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Malignanthypertensionwithararecomplicationofpulmonaryalveolarhemorrhage.A34-year-oldJapanesemalewithseverehypertension,rapidlyprogressiverenalfailure,blurredvision,dyspneaandhemoptysis.Clinicaldiagnosisofmalignanthypertensionwasgivenandantihypertensivetherapyandhemodialysiswereimmediatelystarted.Renalfunctionwasgraduallyrecoveredandpulmonaryhemorrhagecompletelydisappearedbytreatmentwithantihypertensiveagents.Typicalpathologicalchangesofmalignanthypertension,i.e.fibrinoidnecrosisoftheafferentarteriolesandproliferativeendoarteritisattheinterlobulararterieswereobserved.Theauthorsspeculatethatalveolarhemorrhagemayberelatedtovascularinjuriesatthealveolarcapillarylevelcausedbymalignanthypertension.AmJNephrol.2000Jan-Feb;20(1):64-7.

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Themechanismoflunghemorrhageinmalignanthypertension（1）Therearefewreportsoflunghemorrhageinmalignanthypertension.Thepathophysiologyofmalignanthypertensioninvolvesvascularorendothilialinjuriescausedbyseverehypertension.Notonlythemechanicalstressofthehighbloodpressurebutalsoother