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1、Bilingual case discussion2015-09-1112021/7/20 星期二 A 35-year-old man with dyspnea,anemia,and renal failure22021/7/20 星期二several weeks ago,the patient felt fatigue.Two days before admission,dyspnea developed,decreased urination,without fever,cough,or chest pain,no hemoptysis咯血,chills,night sweats,head
2、aches,visual loss,dry eyes,dry mouth,or joint pain.He was transferred by ambulance to the emergency department at this hospital 32021/7/20 星期二Four years before admission,he had been evaluated because of back pain,fatigue,and a temperature of 38.6C.Urinalysis showed hematuria血尿 and proteinuria；testin
3、g for rapid plasma reagin(RPR快速血浆反应素)was positive for antibodies to Treponema pallidum（梅毒螺旋体）.During the previous 6 months,the patient had had episodes of bilateral finger,ankle,and facial swelling,without pain or change in color.He had no history of recent travel,exposure to sick persons,blood tran
4、sfusions,or previous surgery.He did not smoke,drink alcohol,or use illicit drugs.42021/7/20 星期二 On examination,T 36.9C,BP 173/89 mm Hg,P 95b/m,R36 b/m,and S02 88%(ambient air).The skin and conjunctivae 结膜were pale,and there were hypopigmented macules 色素减退斑on the right temple and both lower cheeks an
5、d hyperpigmented macules 色素沉着斑on the bridge of the nose.There were bibasilar rales双肺底湿罗音 in the lungs,and the remainder of the examination was normal.52021/7/20 星期二62021/7/20 星期二72021/7/20 星期二82021/7/20 星期二 total and direct bilirubin胆红素,liver-function tests and lactate were normal.testing for rheuma
6、toid factor,screening of the blood and urine for toxins were negative;Testing for hepatitis B and C viruses and autoantibodies against histones组蛋白 was negative.Serum protein electrophoresis 电泳revealed a diffuse increase in the IgG level.92021/7/20 星期二 Transthoracic cardiac ultrasonography revealed n
7、ormal global cardiac function and right-ventricular size,no evidence of a pericardial effusion心包积液,and findings that were consistent with pulmonary Edema.Ultrasonography of the abdomen revealed normal renal size,position,and echotexture回声特性 and normal arterial blood flow.An electrocardiogram(An elec
8、trocardiogram(ECGECG)showed sinus)showed sinus tachycardia,counterclockwise rotationtachycardia,counterclockwise rotation逆钟向逆钟向,and,and nonspecificnonspecific ST-segment and T-wave abnormalities.ST-segment and T-wave abnormalities.102021/7/20 星期二on admission 112021/7/20 星期二122021/7/20 星期二ground-glas
9、s opacities GGO132021/7/20 星期二142021/7/20 星期二Bronchoscopic examination revealed thick,red mucus in the main-stem and right-lower-lobe bronchi;airways of the left lung were normal.Bronchoalveolar lavage on the right,with 300 and 24,500 red cells per cubic millimeter(in the first tube),975 and 1475 wh
10、ite cells per cubic millimeter(in fourth tubes)In the fourth tube,the white-cell differential count revealed 84%leukocytes白细胞.On the second day,the sputum culture grew very few klebsiella克雷伯 152021/7/20 星期二 what do you think 162021/7/20 星期二Pulmonary hemorrhage？172021/7/20 星期二PCP 卡氏肺孢子？（梅毒阳性、HIV）pulm
11、onary embolism 肺栓塞？182021/7/20 星期二 Rapidly progressive glomerulonephritis Pulmonary hemorrhage192021/7/20 星期二 Ernest W.Goodpasture reported in 1919 on the autopsy findings in the case of an 18-year-old man who had died of massive lung hemorrhage and crescentic glomerulonephritis during the height of
12、 the influenza pandemic The term Goodpastures syndrome is applied to the combination of lung purpura and nephritis,regardless of the underlying pathogenesis.202021/7/20 星期二(一)anti-GBM diseaseantiglomerular basement membrane(GBM)antibodies(anti-GBM disease)anti-GBM antibody 212021/7/20 星期二（二）vasculit
13、isvasculitis大血管炎 大动脉炎 巨细胞动脉炎中等血管炎 结节性多动脉炎 川崎病小血管炎 ANCA相关性血管炎 显微镜下型多血管炎（MPA）肉芽肿性多血管炎（GPA，Wegeners）嗜酸细胞性肉芽肿性多血管炎（EGPA，CSS）免疫复合物相关性小血管炎 冷球蛋白血管炎 IgA血管炎 低补体荨麻疹性血管炎（抗C1q血管炎）222021/7/20 星期二（三）infectionA variety of both systemic and pulmonary microbial infections can be accompanied by pulmonary hemorrhage a
14、nd renal disease,including nephritis.In one exceptional case,legionnaires disease军团菌病.No infections could be implicated in this patient.232021/7/20 星期二242021/7/20 星期二The antinuclear antibody titer was positive at 1:1280and had a homogeneous pattern.The titer forantibodies to double-stranded DNA was
15、positiveat 1:80.252021/7/20 星期二 clinical+imaging+pathology262021/7/20 星期二272021/7/20 星期二 肾小体结构模式图肾小体结构模式图 282021/7/20 星期二292021/7/20 星期二*足细胞足细胞（podocytepodocyte）有初、次级突起，其次级突起间相互嵌合为有初、次级突起，其次级突起间相互嵌合为栅栏状，之间有裂孔，上有裂孔膜。栅栏状，之间有裂孔，上有裂孔膜。302021/7/20 星期二滤过膜滤过膜 filtrationfiltration membranemembrane 又称滤过屏障又称滤
16、过屏障 filtration barrierfiltration barrier，由有由有孔毛细血管内皮、基膜和足细胞裂孔膜构成。孔毛细血管内皮、基膜和足细胞裂孔膜构成。312021/7/20 星期二 滤过屏障模式图滤过屏障模式图 返回322021/7/20 星期二diffusely thickened capillarywalls and mild endocapillary proliferationCellular crescents 332021/7/20 星期二(arrows)tubular atrophy and interstitial inflammationfragmented red cellsThe GBM was markedly thickened,imparting a“wire loop”appearance白金耳342021/7/20 星期二abundant granular immune-complex deposition in the mesangium and along the glomerular basement membrane in a
