机化性肺炎2021.ppt

Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,*,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,*,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,Click to edit Master title style,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,机化性肺炎,机化性肺炎的概念,机化性肺炎是指肺泡和肺泡管中存在肉芽组织栓的一组疾病,由成纤维细胞、肌成纤维细胞、疏松结缔基质、胶原组成，肉芽组织栓可以延伸到细支气管,1835,年,Reynaud,就对此病的组织学改变进行了描述,1901,年,Lange,再次对此病的临床病理学本质进行了描述,1983,年,Davison,等引入了“隐源性机化性肺炎,(cryptogenic organizing pneumonia,1985,年,Epler,将其命名为“闭塞性细支气管炎伴机化性肺炎,(BOOP),2002,年美国胸科协会,/,欧洲呼吸病学会建议将,特发性的机化性肺炎命名为,COP,，与其它疾病相关的机化性肺炎则,称为继发性机化性肺炎,OP,的病理学,Semin Respir Crit Care Med 2012;33:462475.,OP,流行病学,COP,发病率：,1.10/10,万,SOP,发病率：,0.86/10,万,OP,总发病率：,1.96/10,万,5060,岁多见,Thorax 2006;61:805808,机化性肺炎的形成,肺泡上皮损伤引起血浆蛋白包括凝血因子及炎症细胞渗出至肺泡腔,(,渗出期,),活化凝血过程，纤维蛋白沉积,(,凝血期,),机化为肺泡内纤维,-,炎症肉芽,（机化期）,炎症消退，多数基质吸收,(,吸收期,),机化性肺炎的种类,COP,继发性,OP,与其它疾病并存的病理,继发性,OP,感染,药物毒性,胶原病,吸入致病原,(,可卡因,),吸入有害气体,胃食道返流,器官移植,放疗,射频,与免疫相关的疾病,继发于结缔组织病,多肌炎,-,皮肌炎,类风湿性关节炎,干燥综合征,SLE,Polymyalgia rheumatica,系统性硬化症,白塞氏病,继发于结缔组织病,强直性脊柱炎,混合型,CTD,继发于免疫异常疾病,各种常见免疫缺陷综合征,原发性混合丙种球蛋白血症,与感染相关疾病,细菌,肺炎链球菌,军团菌,肺炎支原体,Coxiella burnetti,Nocardia asteroides,肺炎衣原体,金黄色葡萄球菌,寄生虫,Plasmodium vivax,病毒,腺病毒,巨细胞病毒,流感与副流感病毒,HIV,疱疹病毒,真菌,新型隐球菌,PCPPneumocystis jiroveci,其它疾病,吸入性肺炎,乳腺癌放疗,器官移植,骨髓移植,肺移植,肾脏移植,肝移植,药物相关性,其它,炎性肠病,原发性胆汁肝硬化,多发性结节动脉炎,慢性甲状腺炎,血液恶性疾病（,MDS,）,T-,细胞白血病,淋巴瘤,冠状动脉搭桥手术,环境暴露（纺织印染染色、家用火、可卡因）,Sweets,综合征,药物引起,OP,最常见：,胺碘酮，博来霉素，卡马西平，干扰素，金制剂,相对少见,:,醋丁洛尔，阿霉素，,5-,氨基水杨酸，柳氮磺胺嘧啶，呋喃妥因，西罗莫司,罕见Rare:,二性霉素,B,，布西拉明，白消安，瘤可宁,头孢拉定,厄洛替尼,氟伐他丁,L-,色氨酸,米诺环素,尼鲁米特,苯妥英钠,利塞膦酸钠,利妥昔单抗,他克莫司,替莫唑胺,沙利度胺,噻氯匹啶,曲妥珠单抗,硫戊巴比妥,OP,实际上是机体对外来刺激的一种反应形式,找不出原因的,-COP,有原因的,-SOP,药物反应,结蹄组织疾病相关,恶性肿瘤,免疫缺陷综合征,脏器移植,肺脏放疗后,等等。,强的松返回20 mg/d,持续12 weeks,后逐渐减量,PCPPneumocystis jiroveci,其它：感染性肺炎、结核或非结核性分支杆菌感染、肉芽肿并血管炎（韦格氏肉芽肿）、弥漫性肺泡出血、多发性肺梗塞,Plasmodium vivax,58%的患者复发大于1次（平均2.,原发与继发COP复发率无差别；,COP与继发性OP比较-BALF,无较好的关于复发的预测指标；,Alveolar opacity/RD照射区,如复发时激素剂量15-30mg强的松，复诊病理,阿奇霉素250mgbid x 2m,250mg qd 1m,1985年Epler将其命名为“闭塞性细支气管炎伴机化性肺炎(BOOP),1901年Lange再次对此病的临床病理学本质进行了描述,pp 571577,2000,强的松剂量 20 mg/d,CTD-OP与COP的比较,起病方式：,多为亚急性起病，病情较轻；偶有急性起病者临床表现同,ARDS,呼吸系统症状和体征：,包括咳嗽、气促、咯血、胸痛、肺部细湿罗音等，无哮鸣音；,全身症状和体征：,包括低热、盗汗、乏力等，不出现杵状指。,临床表现,缺乏特征性,干咳,(70%),轻度呼吸困难,(65%),体重下降,(60%),OP,的影像,典型影像,斑片状肺泡浸润影（典型,COP,）,不常见影像,孤立性阴影（局灶性,COP,）,浸润性阴影（浸润性,COP,）,少见影像,反晕轮征,进行性肺纤维化并网格及实变,多发性结节,支气管中央型实变,不规则线或带,小叶周围型阴影,Semin Respir Crit Care Med 2012;33:462475.,多灶性实变影,孤立性病灶,支气管中央型,结节型,Eur Radiol(2002)12:14861496,带样改变,纤维化型,反晕轮征,reverse halo,多态性,多发性,多变性,多复发性,少蜂窝肺,影像学特点,OP,影像学的鉴别诊断,多发型片状肺泡影,嗜酸性粒细胞肺炎（慢性）,肺泡细泡癌,-,肺炎型,原发性肺淋巴瘤,吸入性肺炎,其它：感染性肺炎、结核或非结核性分支杆菌感染、肉芽肿并血管炎（韦格氏肉芽肿）、弥漫性肺泡出血、多发性肺梗塞,孤立性灶性结节或肿块,肺癌,圆形肺炎,炎性假瘤,其它：所有原因的结节或肿块疾病,米满星浸润影（进行性,/,致纤维化型,COP,）,IIP,，特别,NSIP,和,AE-IPF,OP,与间质性肺炎并存,其它：所有其它原因浸润影，特别是感染与肿瘤性病变,Semin Respir Crit Care Med 2012;33:462475.,C,临床,R,P,影像学,病理,COP,诊断,CTD-OP,与,COP,的比较,Rheumatology 2011;50:932938,COP,与继发性,OP,比较,-,一般资料,CHEST 2011;139(4):893900,COP,与继发性,OP,比较,-,影像学,CHEST 2011;139(4):893900,COP,与继发性,OP,比较,-,实验室检查,CHEST 2011;139(4):893900,例数（,COP/,继发,OP)OP,+,SD,COP,+,SD,Secondary OP,+,SD,P,Value,Macrophages(27/5),43.94,+,14.28,44.30,+,14.28,42.00,+,15.81,NS,Lymphocytes(27/5),18.84,+,12.19,16.07,+,9.025,33.80,+,17.06,.002,Neutrophils(27/5),26.09,+,16.63,27.52,+,16.36,18.40,+,17.78,NS,Eosinophils(27/5),10.19,+,9.50,11.22,+,9.79,4.60,+,5.50,NS,Mast cells(27/5),2.28,+,2.59,2.52,+,2.68,1.00,+,1.73,NS,Lymphocytes.20%,14(43.8),10(37),4(80),NS,COP,与继发性,OP,比较,-BALF,COP,与继发性,OP,的,PFT,比较,CHEST 2011;139(4):893900,放射性肺炎与,OP,Oie et al.Radiation Oncology 2013,8:56,RP,OP,RT,期间或,RT,结束后不久,RD,结束可达,1,年后,Alveolar opacity/RD,照射区,肺浸润,/,放射区外，呈游走性，可在对侧肺出现,发生率较高,发生率低常有肺纤维化，无复发,激素治疗后吸收，无肺纤维化，停激素后复发,放射性肺炎与,OP,的区别,Oie et al.Radiation Oncology 2013,8:56,OP,复发,初次复发者，,68%,仍然在服用初始治疗的激素,32%,停用激素,2,月,初次复发者，,强的松剂量,20 mg/d,Am J Respir Crit Care Med Vol 162.pp 571577,2000,OP,复发与非复发者比较,-BAL,与肺功能,复发,COP,特点,Human Pathology(2014)45,342351,COP,病情演变,纤维蛋白与,OP,复发,Human Pathology(2014)45,342351,累及肺叶数目与复发,Human Pathology(2014)45,342351,累及肺叶数,+,纤维素与复发,Human Pathology(2014)45,342351,OP,的治疗,激素疗法,强的松,0.75-1.5 mg/kg/d x 4-6,周，或,0.50-0.75 mg/kg/d,每,3-4,周渐减量至,10mg/d,维持,6-12,月,增高剂量不降低复发或提高疗效，增加副作用,重症患者可用激素冲击疗法加强龙,0.5-1g/d x3-5d,Am J Med Sci 2008;335(1):3439.,OP,复发的治疗,激素停药后复发，其中约近患者有多次复发,强的松返回,20 mg/d,持续,12 weeks,后逐渐减量,如,OP,复发单纯基于影像学，无症状与炎症指标反复，密切观察，不建议增加激素剂量,如复发时激素剂量,15-30mg,强的松，复诊病理,Cordier JF.,Clin Chest,Med,2004;25:727738,Rev Port Pneumol.,2011,17(4):186-9,Am J Respir Crit Care Med 2000;162(2 Pt 1):571577,Chest 2005;128(5):36113617,COP,的激素治疗方案,Semin Respir Crit Care Med 201233:462475.,其它疗法,免疫抑制剂,CTX,、硫唑嘌呤：持续、,CT,网格阴影为主、致纤维化型,大环内酯类：阿奇霉素、克拉霉素，辅助治疗,复发、激素不耐受、激素减量,Respir Med 1997;91(3):175177,Pathology International,2012;,62,:144148,Ann Am Thorac Soc.2014 Jan;11(1):87-91,Chest.,2005;128(5):3611-3617,Ann Am Thorac Soc Vol 11,No 1,pp 8791,Jan 2014,阿奇霉素治疗,OP,阿奇霉素,250mgbid x 2m,250mg qd 1m,Chest.,2005;128(5):3611-3617,COP vs CTD-OP,治疗反应,Rheumatology 2011;50:932-938,有一定的自然缓解率；,1358%,的复发率，但复发并不增加死亡率；,58%,的患者复发大于,1,次（平均,2.42.2,）；,68%,在减量期间复发；,强的松剂量在,127mg/d,的范围内易于复发；,1,年内的复发占,82%,；,原发与继发,COP,复发率无差别；,无较好的关于复发的预测指标；,关于预后,Alasaly K,Muler N,Ostrow D,Champion P,FitzGerald JM.Cryptrogenic Organizing Pneumo,nia:A report of 25 cases and a review of literature.Medicine 1995;74:201-211,.,Am J Respir Crit Care Med.2000 Aug;162(2 Pt 1):571-7.,