1、Bilingual case discussion 2015-09-11 A 35-year-old man with dyspnea,anemia,and renal failure 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,headaches,visual loss,dry eyes
2、,dry mouth,or joint pain.He was transferred by ambulance to the emergency department at this hospital 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；testing for rapid plasma reagin(RPR快速血浆反应素)was
3、 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 transfusions,or previous surgery.He did not
4、smoke,drink alcohol,or use illicit drugs.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 and hyperpigmented macules 色素沉着斑on the bridge of the nose
5、.There were bibasilar rales双肺底湿罗音 in the lungs,and the remainder of the examination was normal.total and direct bilirubin胆红素,liver-function tests and lactate were normal.testing for rheumatoid factor,screening of the blood and urine for toxins were negative;Testing for hepatitis B and C viruses and
6、autoantibodies against histones组蛋白 was negative.Serum protein electrophoresis 电泳revealed a diffuse increase in the IgG level.Transthoracic cardiac ultrasonography revealed normal global cardiac function and right-ventricular size,no evidence of a pericardial effusion心包积液,and findings that were consi
7、stent 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(ECG)showed sinus tachycardia,counterclockwise rotation逆钟向,and nonspecific ST-segment and T-wave abnormalities.on admission ground-glas
8、s opacities GGO 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 white cells per cubic millimete
9、r(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 what do you think Pulmonary hemorrhage？PCP 卡氏肺孢子？（梅毒阳性、HIV）pulmonary embolism 肺栓塞？Rapidly progressive glomerulonephritis Pulmonary hemorrh
10、age 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 the influenza pandemic The term Goodpastures syndrome is applied to the combination of lung purpura and n
11、ephritis,regardless of the underlying pathogenesis.(一)anti-GBM disease antiglomerular basement membrane(GBM)antibodies(anti-GBM disease)anti-GBM antibody （二）vasculitis vasculitis 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
12、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）（三）infection A variety of both systemic and pulmonary microbial infections can be accompanied by pulmonary hemorrhage and renal disease,including nephritis.In one exceptional case,legionnaires disease军团菌病.No infections could be implicated in this patient.The antin
13、uclear antibody titer was positive at 1:1280 and had a homogeneous pattern.The titer for antibodies to double-stranded DNA was positive at 1:80.clinical+imaging+pathology 肾小体结构模式图肾小体结构模式图 *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。滤过膜滤过膜 filtratio
14、nfiltration membranemembrane 又称滤过屏障又称滤过屏障 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。滤过屏障模式图滤过屏障模式图 返回 diffusely thickened capillary walls and mild endocapillary proliferation Cellular crescents (arrows)tubular atrophy and interstitial inflammation fragmented r
15、ed cells The GBM was markedly thickened,imparting a“wire loop”appearance 白金耳 abundant granular immune-complex deposition in the mesangium and along the glomerular basement membrane in a classic“full house”pattern 满堂亮(anti-IgG immunofluorescence)Immunecomplex deposition is also seen along the tubular
16、 basement membrane abundant electron-dense deposits are seen in a mesangial,intramembranous,subepithelial,and subendothelial distribution showed strong staining with IgG,IgM,IgA,C3,C1q,and kappa and lambda light chains in a granular pattern in the mesangium and along the GBM 所涉及的病理学术语的定义 弥漫性病变(diffuse)(diffuse)：病变累及50肾小球。局灶性病变(focal)(focal)：病变仅累及50肾小球。球性病变(global)(global)：病变累及一个肾小球的大部分毛细血管袢(50)。节段性病变(segmental)(segmental)：病变仅累及一个肾小球的少部分毛细血管袢(50)。系膜细胞增生(mesangial hypercellularity)(mesangial hyper