恶性高血压与肺出血

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1、IgA肾病、恶性高血压与肺出血北京协和医院1IgA肾病与肺出血2Pulmonary capillaritis and alveolar hemorrhage.Update on diagnosis and management.nPulmonaryvascularinflammatorydisordersmayinvolveallcomponentsofthepulmonaryvasculature,includingcapillaries.nTheprincipalhistopathologicfeaturesofpulmonarycapillaritisincludecapillarywa

2、llnecrosiswithinfiltrationbyneutrophils,interstitialerythrocytes,and/orhemosiderin,andinteralveolarseptalcapillaryocclusionbyfibrinthrombi.Immunecomplexdepositionisvariablypresent.nPatientsoftenpresentclinicallywithdiffusealveolarhemorrhage,whichischaracterizedbydyspneaandhemoptysis;diffuse,bilatera

3、l,alveolarinfiltratesonchestradiograph;andanemia.nPulmonarycapillaritishasbeenreportedwithvariablefrequencyandseverityasamanifestationofWegenersgranulomatosis,microscopicpolyarteritis,systemiclupuserythematosus,Goodpasturessyndrome,idiopathicpulmonaryrenalsyndrome,Behcetssyndrome,Henoch-Schonleinpur

4、pura,IgAnephropathy,antiphospholipidsyndrome,progressivesystemicsclerosis,anddiphenylhydantoinuse.3A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation.n34patientswithbiopsy-confirmeddiffusepulmonaryhemorrhage(DPH).nDPHsyndromeswereencountered:antibasem

5、entmembraneantibodydisease(fourcases);idiopathicpulmonaryhemorrhage(fourcases);WG(fivecases);probableWG(sixcases);systemicnecrotizingvasculitisotherwiseunclassified(threecases);systemiclupuserythematosus(twocases);rheumatoidarthritis(onecase);seronegativejuvenilerheumatoidarthritis(onecase);IgAnephr

6、opathy(onecase);idiopathicglomerulonephritis(twocases-onewithandonewithoutimmunecomplexes);andunclassifiedpulmonary-renalsyndromes(fivecases).nCapillaritiswasfoundinlungbiopsysamplesfrom30ofthe34patients(88%)andincludedpatientswitheverytypeofDPHsyndrome.AmJSurgPathol.1990Dec;14(12):1112-25.4Pulmonar

7、yhemorrhage.AfatalmanifestationinIgAnephropathy.nTwopatientswithasymptomaticIgAnephropathy(IgAN)andathirdpatientwithchronicrenalfailureduetoIgANdiedfollowingarecentonsetofdyspnea,hemoptysis,andpulmonaryinfiltrates.nInallcases,thecauseofdeathwasrespiratoryfailureattributedtoeitherbronchopneumoniaorpu

8、lmonaryedema.nInallthreepatients,thediagnosesofIgANandidiopathicpulmonaryhemorrhagewereestablishedatpostmortemexamination.Acutealveolarhemorrhagewaspresentintwopatients.Allthreepatientshadheavyalveolarhemosiderin-ladenmacrophages,andcapillaritiswasrecognizedintwoofthem.nTheysuggestedanimmunecomplex-

9、mediatedpulmonaryinjurythatwaspossiblyrelatedtothesystemicnatureofIgAN.ArchPatholLabMed.1994May;118(5):542-6.5IgA肾病与恶性高血压6恶性高血压的临床特征(1)血压明显升高,通常舒张压130 mmHg(16.9 kPa);(2)广泛累及全身小动脉,导致中枢神经系统、心、肾等脏器受损,其中以肾脏损害最为显著;(3)眼底改变包括视网膜出血、棉絮状渗出及视乳头水肿等;(4)如不及时治疗则预后不佳,多死于尿毒症。7恶性高血压的病因89Prognostic factors in immunogl

10、obulin-A nephropathynNinety eight adult patients with diagnosis of primary IgA nephropathy.nOut of 98 patient，64(65.3%)were men.Mean age of presentation was 25.7 years.nThe predominant renal lesions included nephrotic syndrome in 25(25.5%),rapidly progressive renal failure and accelerated malignant

11、hypertension in 21(21.4%)each,chronic renal failure in 13(13.3%),hypertension in nine(9.2%)haematuria in five(5.1%)and acute renal failure in four(4.1%).Sixty(61%)had renal failure at diagnosis.Age 25 years,glomerular histology of Hass subclass V and interstitial fibrosis were significant factors.Fo

12、rty(48.2%)(IR)patients developed ESRF during follow up.JAssocPhysiciansIndia.2002Nov;50:1354-9.10Malignant or accelerated hypertension in IgA nephropathy.n66 adult patients diagnosed as having IgA nephropathy by renal biopsy n24(36%)were hypertensive when first seen.Of these hypertensive patients,10

13、(15%)had malignant or accelerated hypertension.nAll patients but one were male and had no knowledge of their renal disease and sought medical advice for symptoms due to hypertension.Five patients had no history of gross hematuria.nHistological vascular findings showed,in three proliferative endarter

14、itis and fibrinoid necrosis,in five arteriolosclerosis and in two vascular hypertrophy.In spite of good blood pressure control,six patients reached terminal uremia within a maximum of 14 months.nPatients with this association reach end stage renal failure in a short period of time.ClinNephrol.1987Ja

15、n;27(1):1-7.11“Malignant”IgAnephropathy（1）nMostpatientswithmesangialIgAnephropathywhorunaprogressivecourseusuallydosooveraperiodof10to20years.nThispaperdescribesthecourseofthreeyoungmenwithsimilarpresentingfeaturesandbiopsyfindingswhoprogressedtoend-stagerenalfailureinlessthan4yearsfrompresentation,

16、eventhoughinitiallyallhadserumcreatininelevelsthatwereinthenormalrange.AmJKidneyDis.1985Jan;5(1):42-6.12“Malignant”IgAnephropathy（2）nTheypresentedwithmacroscopichematuria,whichhaspreviouslybeenregardedasanindicatorofafavorableprognosis,andallthreehadloinpain,constantlyelevatedurinaryerythrocytecount

17、s,andcrescentsinrenalbiopsies.nIntwocases,treatmentappearedtobeassociatedwithstabilizationofrenalfunction,butdeteriorationtoend-stagerenalfailureoccurredrapidlyaftertreatmentwasceased.AmJKidneyDis.1985Jan;5(1):42-6.13Idiopathic IgA nephropathy presenting as malignant hypertension.nThreecasesarerepor

18、tedofidiopathicIgAnephropathy(Bergersdisease)presentingasmalignanthypertension,withnodatasuggestingtheunderlyingglomerulopathy,whichwasuncoveredonlyafterrenalbiopsywasperformed.Commentsaremadeonthevalidityofthepathologicaldiagnosis,thepossiblepathogeneticsequenceoftheassociation,andontheeventualrisk

19、sandbenefitsderivedfromperformingrenalbiopsyinsuchpatients.AmJNephrol.1986;6(6):482-6.14恶性高血压与肺出血15张晓亮查房复习n男，35岁，C800489入院日期：2003-8-1n主主诉诉：双小腿水肿1年，咯血5月，憋气3月，加重10余天。16现病史现病史n1年前无明显诱因出现双小腿可凹性水肿。间断头痛，未测血压。n5月前咳少量痰，带血丝，逐渐加重有小血块。并出现憋气，夜尿增多，夜间尿3-4次，尿量无减少，尿中泡沫增多，双下肢肿较前明显加重。n03-6月初憋气加重，夜间不能平卧，双小腿水肿明显，晨起后自觉眼

20、睑浮肿，并出现皮肤、巩膜较前变黄。17第一次住院情况n查体：血压240/130 mmHg,巩膜黄染，双下肢可凹性水肿（+）n尿常规PRO 5g/L，BLD 250/UL；尿蛋白 g/d，GFR22.7ml/min，免疫指标（-）。6-10血气：I型呼衰。6-16肾穿“恶性高血压肾损害”；胸片、胸部CT：双肺多发渗出实变性病变，右侧包裹性胸腔积液；肺V/Q显像：不支持肺栓塞。心脏彩超：左室肥厚，左房增大，射血分数57%，轻度肺动脉高压。n入院后Scr增高，mg/dl，6-15开始甲强g/d*3天冲击，后改为强的松60mg/日。n呼吸科会诊考虑咯血为高血压所致。予降压药治疗后，血压控制至140/9

21、0mmHg，憋气等症状缓解，强的松减至15mg/天，后6-30出院。n出院后半月内逐渐停用强的松。18第二次住院情况n7月18日再次出现头痛、憋气、痰中带血，浮肿加重，夜间不能平卧。急诊查T38.2，BP185/87mmHg，SCr5.8mg/dl，胸片：双肺大片高密度影，肺水肿可能；血气：PO245.8mmHg，SO283.6%，予CPAP辅助呼吸及抗感染治疗，持续硝普钠、压宁定等控制血压。n肺活检示肺左下叶基底段慢性炎，肺泡腔内有较多吞噬含铁黄素的吞噬细胞。n卧位PRA9.4mug/ml，AT409.6uug/ml，Ald21.7mug/dln24小时尿CA：NE16.43ug/d；E3.

22、43ug/d;DA48.77ug/dn24小时尿UFC57.8ug/d19诊断诊断：恶性高血压 急性肾功能衰竭 急性左心功能衰竭 高血压视网膜病变期 肺内病变性质未明 型呼吸衰竭 肺水肿 肺泡出血合并感染可能性大 肺结核不除外 2型糖尿病 结节性甲状腺肿20Malignant hypertension with a rare complication of pulmonary alveolar hemorrhage.nA 34-year-old Japanese male with severe hypertension,rapidly progressive renal failure,bl

23、urred vision,dyspnea and hemoptysis.Clinical diagnosis of malignant hypertension was given and antihypertensive therapy and hemodialysis were immediately started.nRenal function was gradually recovered and pulmonary hemorrhage completely disappeared by treatment with antihypertensive agents.nTypical

24、 pathological changes of malignant hypertension,i.e.fibrinoid necrosis of the afferent arterioles and proliferative endoarteritis at the interlobular arteries were observed.n The authors speculate that alveolar hemorrhage may be related to vascular injuries at the alveolar capillary level caused by

25、malignant hypertension.AmJNephrol.2000Jan-Feb;20(1):64-7.21The mechanism of lung hemorrhage in malignant hypertension（1）nTherearefewreportsoflunghemorrhageinmalignanthypertension.nThepathophysiologyofmalignanthypertensioninvolvesvascular or endothilial injuries caused by severehypertension.nNotonlyt

26、hemechanicalstressofthehighbloodpressurebutalsootherhumoralfactors,suchasrenin,aldosterone,vasopressin,cathecholamines,endothelinandcoagulationfactorsmaybeinvlovedinthisprocess.22The mechanism of lung hemorrhage in malignant hypertension（2）nInsmallarteries,thedenudationofendotheliumcausesplateletadh

27、erence,releaseofPDGFandproliferationofsmoothmusclecellsandthusresultinmusculomucoidintimalhyperplasia.nInarterioles,theincreasedpermeabilityofendotheliumcausestheextravasationoffibrinogenanddepositionoffibrin,andfinallyresultsinnecrosisofsmoothmusclecells.23The mechanism of lung hemorrhage in malignant hypertension（3）nIn the retinal capillaries,thickening,hyalinization andocclusionareobserved.nIn glomerular capillaries,there is wrinking andduplicationofglomerularbasementmembranes.nTherarecomplicationofalveolarhemorrhagemagbecausedbytheinjuriesofalveolarcapillaries.24