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1、1,IgA肾病、恶性高血压与肺出血,北京协和医院,2,IgA肾病与肺出血,3,Pulmonary capillaritis and alveolar hemorrhage.Update on diagnosis and management.,Pulmonary vascular inflammatory disorders may involve all components of the pulmonary vasculature,including capillaries.The principal histopathologic features of pulmonary capill
2、aritis include capillary wall necrosis with infiltration by neutrophils,interstitial erythrocytes,and/or hemosiderin,and interalveolar septal capillary occlusion by fibrin thrombi.Immune complex deposition is variably present.Patients often present clinically with diffuse alveolar hemorrhage,which i
3、s characterized by dyspnea and hemoptysis;diffuse,bilateral,alveolar infiltrates on chest radiograph;and anemia.Pulmonary capillaritis has been reported with variable frequency and severity as a manifestation of Wegeners granulomatosis,microscopic polyarteritis,systemic lupus erythematosus,Goodpastu
4、res syndrome,idiopathic pulmonary renal syndrome,Behcets syndrome,Henoch-Schonlein purpura,IgA nephropathy,antiphospholipid syndrome,progressive systemic sclerosis,and diphenylhydantoin use.,4,A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation.,34 pat
5、ients with biopsy-confirmed diffuse pulmonary hemorrhage(DPH).DPH syndromes were encountered:antibasement membrane antibody disease(four cases);idiopathic pulmonary hemorrhage(four cases);WG(five cases);probable WG(six cases);systemic necrotizing vasculitis otherwise unclassified(three cases);system
6、ic lupus erythematosus(two cases);rheumatoid arthritis(one case);seronegative juvenile rheumatoid arthritis(one case);IgA nephropathy(one case);idiopathic glomerulonephritis(two cases-one with and one without immune complexes);and unclassified pulmonary-renal syndromes(five cases).Capillaritis was f
7、ound in lung biopsy samples from 30 of the 34 patients(88%)and included patients with every type of DPH syndrome.Am J Surg Pathol.1990 Dec;14(12):1112-25.,5,Pulmonary hemorrhage.A fatal manifestation in IgA nephropathy.,Two patients with asymptomatic IgA nephropathy(IgAN)and a third patient with chr
8、onic renal failure due to IgAN died following a recent onset of dyspnea,hemoptysis,and pulmonary infiltrates.In all cases,the cause of death was respiratory failure attributed to either bronchopneumonia or pulmonary edema.In all three patients,the diagnoses of IgAN and idiopathic pulmonary hemorrhag
9、e were established at postmortem examination.Acute alveolar hemorrhage was present in two patients.All three patients had heavy alveolar hemosiderin-laden macrophages,and capillaritis was recognized in two of them.They suggested an immune complex-mediated pulmonary injury that was possibly related t
10、o the systemic nature of IgAN.Arch Pathol Lab Med.1994 May;118(5):542-6.,6,IgA肾病与恶性高血压,7,恶性高血压的临床特征,血压明显升高,通常舒张压 130 mmHg(16.9 kPa);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;眼底改变包括视网膜出血、棉絮状渗出及视乳头水肿等;如不及时治疗则预后不佳,多死于尿毒症。,8,恶性高血压的 病因,9,10,Prognostic factors in immunoglobulin-A nephropathy,Ninety eight ad
11、ult patients with diagnosis of primary IgA nephropathy.Out of 98 patient，64(65.3%)were men.Mean age of presentation was 25.7 years.The predominant renal lesions included nephrotic syndrome in 25(25.5%),rapidly progressive renal failure and accelerated malignant hypertension in 21(21.4%)each,chronic
12、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.Forty(48.2%)(IR)patients developed ESRF
13、during follow up.J Assoc Physicians India.2002 Nov;50:1354-9.,11,Malignant or accelerated hypertension in IgA nephropathy.,66 adult patients diagnosed as having IgA nephropathy by renal biopsy 24(36%)were hypertensive when first seen.Of these hypertensive patients,10(15%)had malignant or accelerated
14、 hypertension.All 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.Histological vascular findings showed,in three proliferative endarteritis and fibrinoid necrosis,in five
15、 arteriolosclerosis and in two vascular hypertrophy.In spite of good blood pressure control,six patients reached terminal uremia within a maximum of 14 months.Patients with this association reach end stage renal failure in a short period of time.Clin Nephrol.1987 Jan;27(1):1-7.,12,“Malignant”IgA nep
16、hropathy（1）,Most patients with mesangial IgA nephropathy who run a progressive course usually do so over a period of 10 to 20 years.This paper describes the course of three young men with similar presenting features and biopsy findings who progressed to end-stage renal failure in less than 4 years f
17、rom presentation,even though initially all had serum creatinine levels that were in the normal range.Am J Kidney Dis.1985 Jan;5(1):42-6.,13,“Malignant”IgA nephropathy（2）,They presented with macroscopic hematuria,which has previously been regarded as an indicator of a favorable prognosis,and all thre
18、e had loin pain,constantly elevated urinary erythrocyte counts,and crescents in renal biopsies.In two cases,treatment appeared to be associated with stabilization of renal function,but deterioration to end-stage renal failure occurred rapidly after treatment was ceased.Am J Kidney Dis.1985 Jan;5(1):
19、42-6.,14,Idiopathic IgA nephropathy presenting as malignant hypertension.,Three cases are reported of idiopathic IgA nephropathy(Bergers disease)presenting as malignant hypertension,with no data suggesting the underlying glomerulopathy,which was uncovered only after renal biopsy was performed.Commen
20、ts are made on the validity of the pathological diagnosis,the possible pathogenetic sequence of the association,and on the eventual risks and benefits derived from performing renal biopsy in such patients.Am J Nephrol.1986;6(6):482-6.,15,恶性高血压与肺出血,16,张晓亮查房复习,男，35岁，C800489 入院日期：2003-8-1主诉：双小腿水肿1年，咯血5
21、月，憋气3 月，加重10余天。,17,现病史,1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。5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。03-6月初憋气加重，夜间不能平卧，双小腿水肿明显，晨起后自觉眼睑浮肿，并出现皮肤、巩膜较前变黄。,18,第一次住院情况,查体：血压240/130 mmHg,巩膜黄染，双下肢可凹性水肿（+）尿常规PRO 5g/L，BLD 250/UL；尿蛋白 4.96g/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、
22、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。入院后Scr增高，1.94.5mg/dl，6-15开始甲强0.5g/d*3天冲击，后改为强的松60mg/日。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140/90mmHg，憋气等症状缓解，强的松减至15mg/天，Cr 3.3mg/dl后6-30出院。出院后半月内逐渐停用强的松。,19,第二次住院情况,7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，SCr 5.8mg/dl，胸片
23、：双肺大片高密度影，肺水肿可能；血气：PO2 45.8mmHg，SO2 83.6%，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。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。卧位PRA 9.4 mug/ml，AT 409.6 uug/ml，Ald 21.7 mug/dl24小时尿CA：NE 16.43ug/d；E 3.43 ug/d;DA 48.77 ug/d24小时尿UFC 57.8 ug/d,20,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
24、型糖尿病 结节性甲状腺肿,21,Malignant hypertension with a rare complication of pulmonary alveolar hemorrhage.,A 34-year-old Japanese male with severe hypertension,rapidly progressive renal failure,blurred vision,dyspnea and hemoptysis.Clinical diagnosis of malignant hypertension was given and antihypertensive t
25、herapy and hemodialysis were immediately started.Renal function was gradually recovered and pulmonary hemorrhage completely disappeared by treatment with antihypertensive agents.Typical pathological changes of malignant hypertension,i.e.fibrinoid necrosis of the afferent arterioles and proliferative
26、 endoarteritis at the interlobular arteries were observed.The authors speculate that alveolar hemorrhage may be related to vascular injuries at the alveolar capillary level caused by malignant hypertension.Am J Nephrol.2000 Jan-Feb;20(1):64-7.,22,The mechanism of lung hemorrhage in malignant hyperte
27、nsion（1）,There are few reports of lung hemorrhage in malignant hypertension.The pathophysiology of malignant hypertension involves vascular or endothilial injuries caused by severe hypertension.Not only the mechanical stress of the high blood pressure but also other humoral factors,such as renin,ald
28、osterone,vasopressin,cathecholamines,endothelin and coagulation factors may be invloved in this process.,23,The mechanism of lung hemorrhage in malignant hypertension（2）,In small arteries,the denudation of endothelium causes platelet adherence,release of PDGF and proliferation of smooth muscle cells
29、 and thus result in musculomucoid intimal hyperplasia.In arterioles,the increased permeability of endothelium causes the extravasation of fibrinogen and deposition of fibrin,and finally results in necrosis of smooth muscle cells.,24,The mechanism of lung hemorrhage in malignant hypertension（3）,In the retinal capillaries,thickening,hyalinization and occlusion are observed.In glomerular capillaries,there is wrinking and duplication of glomerular basement membranes.The rare complication of alveolar hemorrhage mag be caused by the injuries of alveolar capillaries.,
