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1、IgA肾病、恶性高血压与肺出血北京协和医院IgA肾病与肺出血Pulmonary capillaritis and alveolar hemorrhage. Update on diagnosis and management. nPulmonary vascular inflammatory disorders may involve all components of the pulmonary vasculature, including capillaries.n The principal histopathologic features of pulmonary capillarit
2、is 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. nPatients often present clinically with diffuse alveolar hemorrhage, whic
3、h is characterized by dyspnea and hemoptysis; diffuse, bilateral, alveolar infiltrates on chest radiograph; and anemia. nPulmonary capillaritis has been reported with variable frequency and severity as a manifestation of Wegeners granulomatosis, microscopic polyarteritis, systemic lupus erythematosu
4、s, Goodpastures syndrome, idiopathic pulmonary renal syndrome, Behcets syndrome, Henoch-Schonlein purpura, IgA nephropathy, antiphospholipid syndrome, progressive systemic sclerosis, and diphenylhydantoin use. A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy con
5、firmation. n34 patients with biopsy-confirmed diffuse pulmonary hemorrhage (DPH).nDPH 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 uncl
6、assified (three cases); systemic 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
7、syndromes (five cases).n Capillaritis was found in lung biopsy samples from 30 of the 34 patients (88%) and included patients with every type of DPH syndrome. nAm J Surg Pathol. 1990 Dec;14(12):1112-25. Pulmonary hemorrhage. A fatal manifestation in IgA nephropathy. nTwo patients with asymptomatic I
8、gA nephropathy (IgAN) and a third patient with chronic renal failure due to IgAN died following a recent onset of dyspnea, hemoptysis, and pulmonary infiltrates.nIn all cases, the cause of death was respiratory failure attributed to either bronchopneumonia or pulmonary edema. nIn all three patients,
9、 the diagnoses of IgAN and idiopathic pulmonary hemorrhage 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. nThey suggested an immune
10、 complex-mediated pulmonary injury that was possibly related to the systemic nature of IgAN.nArch Pathol Lab Med. 1994 May;118(5):542-6. IgA肾病与恶性高血压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)眼底改动包括视网膜出血、棉絮状渗出及视乳头水肿等; (4)如不及时治疗那么预后不佳,多死于尿毒症。恶性高血压的 病
11、因 Prognostic factors in immunoglobulin-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
12、renal failure and accelerated malignant 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
13、V and interstitial fibrosis were significant factors. Forty (48.2%) (IR) patients developed ESRF during follow up. nJ Assoc Physicians India. 2019 Nov;50:4-9. Malignant or accelerated hypertension in IgA nephropathy.n66 adult patients diagnosed as having IgA nephropathy by renal biopsy n24 (36%) wer
14、e hypertensive when first seen. Of these hypertensive patients, 10 (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.
15、nHistological vascular findings showed, in three proliferative endarteritis 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
16、 reach end stage renal failure in a short period of time.nClin Nephrol. 1987 Jan;27(1):1-7. “Malignant IgA nephropathy1nMost patients with mesangial IgA nephropathy who run a progressive course usually do so over a period of 10 to 20 years.n This paper describes the course of three young men with si
17、milar presenting features and biopsy findings who progressed to end-stage renal failure in less than 4 years from presentation, even though initially all had serum creatinine levels that were in the normal range.n Am J Kidney Dis. 1985 Jan;5(1):42-6.“Malignant IgA nephropathy2nThey presented with ma
18、croscopic hematuria, which has previously been regarded as an indicator of a favorable prognosis, and all three had loin pain, constantly elevated urinary erythrocyte counts, and crescents in renal biopsies.n In two cases, treatment appeared to be associated with stabilization of renal function, but
19、 deterioration to end-stage renal failure occurred rapidly after treatment was ceased.nAm J Kidney Dis. 1985 Jan;5(1):42-6. Idiopathic IgA nephropathy presenting as malignant hypertension. nThree cases are reported of idiopathic IgA nephropathy (Bergers disease) presenting as malignant hypertension,
20、 with no data suggesting the underlying glomerulopathy, which was uncovered only after renal biopsy was performed. Comments 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 re
21、nal biopsy in such patients.nAm J Nephrol. 1986;6(6):482-6. 恶性高血压与肺出血张晓亮查房复习n男，35岁，C800489 入院日期：2019-8-1n主诉：双小腿水肿1年，咯血5月，憋气3 月，加重10余天。 现病史现病史 n1年前无明显诱因出现双小腿可凹性水肿。延续头痛，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，晨起后自觉眼睑浮肿，并出现皮肤、巩膜较前变黄。 第一次住院情况n查体：血
22、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 4.96g/d，GFR22.7ml/min，免疫目的-。 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，1.94.5mg/dl，6-15开场甲强0.5g/d*3天冲击 ，后改为强的松60mg/日。n呼吸科会诊思索咯血为高血压所致。予降压药治疗后，血压控制至140/90mmHg，憋气等病症缓解，强的松减
23、至15mg/天，Cr 3.3mg/dl后6-30出院。n出院后半月内逐渐停用强的松。 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，SCr 5.8mg/dl，胸片：双肺大片高密度影，肺水肿能够；血气：PO2 45.8mmHg，SO2 83.6%，予CPAP辅助呼吸及抗感染治疗，继续硝普钠、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PRA 9.4 mug/ml，AT 409.6 uug/ml，Ald 21.7 mug/dln24小时尿CA：NE 16.43ug/d；
24、E 3.43 ug/d; DA 48.77 ug/dn24小时尿UFC 57.8 ug/d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 肺泡出血合并感染能够性大 肺结核不除外 2型糖尿病 结节性甲状腺肿Malignant hypertension with a rare complication of pulmonary alveolar hemorrhage.nA 34-year-old Japanese male with severe hypertension, rapidly progressive renal f
25、ailure, blurred 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 age
26、nts. nTypical 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 l
27、evel caused by malignant hypertension. nAm J Nephrol. 2000 Jan-Feb;20(1):64-7. The mechanism of lung hemorrhage in malignant hypertension1nThere are few reports of lung hemorrhage in malignant hypertension.nThe pathophysiology of malignant hypertension involves vascular or endothilial injuries cause
28、d by severe hypertension.nNot only the mechanical stress of the high blood pressure but also other humoral factors, such as renin, aldosterone, vasopressin, cathecholamines, endothelin and coagulation factors may be invloved in this process.The mechanism of lung hemorrhage in malignant hypertension2
29、nIn small arteries, the denudation of endothelium causes platelet adherence, release of PDGF and proliferation of smooth muscle cells and thus result in musculomucoid intimal hyperplasia. nIn arterioles, the increased permeability of endothelium causes the extravasation of fibrinogen and deposition
30、of fibrin, and finally results in necrosis of smooth muscle cells.The mechanism of lung hemorrhage in malignant hypertension3nIn the retinal capillaries, thickening, hyalinization and occlusion are observed.nIn glomerular capillaries, there is wrinking and duplication of glomerular basement membranes.nThe rare complication of alveolar hemorrhage mag be caused by the injuries of alveolar capillaries.
