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1、Bilingual case discussion2015-09-11 A 35-year-old man with dyspnea, anemia, and renal failureseveral 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 los
2、s, dry eyes,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
3、(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 transfusions, or previ
4、ous surgery.He did not 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 macu
5、les 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. 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; Te
6、sting 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. Transthoracic cardiac ultrasonography revealed normal global cardiac function and right-ventricular size, no evidence of a pericardial
7、 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 electrocardiogram (ECGECG) showed sinus ) showed sinus tachycardia,counterclockwise
8、 rotationtachycardia,counterclockwise rotation逆钟向逆钟向, and , and nonspecificnonspecific ST-segment and T-wave abnormalities.ST-segment and T-wave abnormalities.on admission ground-glass opacities GGOBronchoscopic examination revealed thick, red mucus in the main-stem and right-lower-lobe bronchi; air
9、ways 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 millimeter (in fourth tubes) In the fourth tube, the white-cell differential count revealed 84% leukocytes白细胞. On the se
10、cond 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 hemorrhage Ernest W. Goodpasture reported in 1919 on the autopsy findings in the case of an 18-year-old man who
11、 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 nephritis, regardless of the underlying pathogenesis.(一)anti-GBM diseaseantiglomerular basement membrane
12、 (GBM) antibodies (anti-GBM disease)anti-GBM antibody （二）vasculitisvasculitis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）（三）infectionA variety of both systemic and pulmonary microbial in
13、fections 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 antinuclear antibody titer was positive at 1:1280and had a homogeneous pattern. The titer forantibodies
14、to double-stranded DNA was positiveat 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 filtrationfiltration 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
15、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 capillarywalls and mild endocapillary proliferationCellular crescents (arrows)tubular atrophy and interstitial inflammationfragmented red cellsThe GBM was markedly thickened, imparting a “wire loop” appearance白金耳abundant granular im
16、mune-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 basement membraneabundant electron-dense depositsare seen in a mesangial, intramembranous, sub
17、epithelial, and subendothelial distributionshowed 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 弥漫性病变弥漫性病变(diffuse)(diffuse)(diffuse)(diffuse)：病变累及50肾小球。 局灶性病变局灶性病变(focal)(focal)(focal)(focal)：病变仅累及
18、50肾小球。 球性病变球性病变(global)(global)(global)(global)：病变累及一个肾小球的大部分毛细血管袢(50)。 节段性病变节段性病变(segmental)(segmental)(segmental)(segmental)：病变仅累及一个肾小球的少部分毛细血管袢(50)。 系膜细胞增生系膜细胞增生(mesangial hypercellularity)(mesangial hypercellularity)(mesangial hypercellularity)(mesangial hypercellularity)：3m切片中，一个系膜区超过3个细胞。 毛细血管
19、内增生毛细血管内增生(endocapillary proliferation)(endocapillary proliferation)(endocapillary proliferation)(endocapillary proliferation)：肾小球毛细血管内皮细胞和系膜细胞增生，单个核细胞浸润，导致毛细血管腔狭窄。I型 轻微病变性狼疮性肾炎II型 系膜增殖性狼疮性肾炎III型 局灶性狼疮性肾炎III（A）：活动性病变局灶增殖性LNIII（A/C）：活动和慢性化病变局灶增殖和硬化性LNIII（C）：慢性非活动性病变局灶硬化性LNIV型 弥漫性狼疮性肾炎IV-S（A）IV-G（A）IV
20、-S（A/C）型 膜性狼疮性肾炎型 终末硬化性狼疮性肾炎狼疮性肾炎的病理组织学分类狼疮性肾炎的病理组织学分类狼疮性肾炎的病理组织学分类狼疮性肾炎的病理组织学分类（ISN/RPS 2003）IV-G（A/C）IV-S（C）IV-G（C）根据活动性和硬化性病变又分为多个亚型IV-S（A）：活动性病变弥漫节段增殖性LNIV-G（A）：活动性病变弥漫球性增殖性LNIV-S（A/C）：活动和慢性化病变并存弥漫节段增殖和硬化性LNIV-G（A/C）：活动和慢性化病变并存弥漫球性增殖和硬化性LNIV-S（C）：慢性非活动性病变伴疤痕形成弥漫节段硬化性LNIV-G（C）：慢性非活动性病变伴疤痕形成弥漫球性硬化
21、性LNIV型型 弥漫性狼疮性肾炎弥漫性狼疮性肾炎SLE with diffuse proliferative lupus nephritis, with active and chronic lesions (class IV-G A/C), and pulmonary alveolar hemorrhage. 梅毒检测 临床上通常用快速血浆反应素环状卡片试验(RPR)筛查梅毒患者。梅毒螺旋体在破坏组织时，释放出心磷脂刺激机体产生抗心磷脂抗体。 RPR用从牛心提取的心磷脂加卵磷脂和胆固醇的组合成分作为抗原。与机体产生的反应素在体外反应。而牛心提取的心磷脂可与患者血清中抗心磷脂抗体发生免疫反应。CTD患者体内同样存在抗心磷脂抗体，因此RPR在CTD中存在假阳性，尤其是SLE、APS。国外报道SLE患者中25一30出现RPR假阳性 Take home massages1.梅毒假阳性原因2.Goodpastures syndrome 3.狼疮肾炎
